Clementovsky – Scleroderma – 1873

The Induration of the Cellular Tissue of the Newborn
[Die Zellgewebsverhärtung der Neugeborenen]

By Dr. A. Clementovsky, Senior Physician of the Imperial Foundling Hospital in Moscow

(Oesterreichisches Jahrbuch für Paediatrik, IV. Jahrgang 1873, Vol. I, pp. 1–48)
Machine-assisted translation from the original German publication.

Since the anatomical types of the various morbid processes are conditioned by the peculiarity of the animal tissues—which, according to their essential nature, are of like kind at every age—one would suppose that the anatomical types of sclerema in children as in adults, in the non-newborn as in the newborn, must be the same. In fact, however, this is not the case, and the induration of the cellular tissue in the latter either does not appear in the familiar form, or else presents such peculiarities as impress upon it a wholly special character. Thus, for example, the fibrous degeneration of the subcutaneous cellular tissue (scleroderma) has as yet been observed by no one in the newborn, while, on the other hand, the most frequent form—the oedematous sclerema—shows in the newborn phenomena such as are never observed in adults. There can be no doubt that a divergence of this kind depends upon the physiological conditions of the infant organism.

Among those forms which present like characters in the non-newborn and in the newborn, the erysipelatous (rose-like) sclerema must be counted.

Erysipelatous Sclerema

This sclerema plainly bears the inflammatory character. The induration of the skin and of the subcutaneous cellular tissue is accompanied at the same time by redness and elevated temperature of the skin, by a generally febrile state, and by painfulness on contact, whereby the affected parts are more or less swollen. This swelling has at times, in certain places—e.g. on the feet—an oedematous character; the colour of the skin shows various nuances, playing from red into bluish. On post-mortem examination the subcutaneous cellular tissue here displays all the peculiarities of so-called inflammatory infiltration and of inflammatory oedema.

When the rose (erysipelas) subsides, the indurated places become paler and at times take on a yellowish-red coloration. Thereupon the desquamation of the epidermis begins, and with it the hardness of the affected parts likewise diminishes, although the latter yet at times persists—indeed remains perceptible even when the skin has already regained its normal coloration. In the further course, however, the hardness also begins to break up and gradually disappears entirely. In this latter case one cannot avoid recognizing the transition of the simple inflammatory infiltrate into the so-called inflammatory hypertrophy, which comes to resolution through fatty involution of the cellular elements. Since the rose of the newborn is frequently a migrating one, the induration of the skin and subcutaneous cellular tissue also participates in this—that is, it appears where the rose appears, either resolving together with the erysipelatous process or persisting for some time in the previously affected parts.

It is chiefly under this form that the erysipelatous sclerema of the newborn has presented itself to my observation; and whether this disease exhibits any deviations from this form is unknown to me.

The literature of erysipelatous sclerema is still exceedingly scanty. In adults the sclerema also cannot appear immediately after the rose, as, for example, in the case described by Plu de Grand-Lucé (Gazette des hôpitaux 1866, No. 77), where the sclerema appeared only a month and a half after the rose.

Oedematous Sclerema

The most frequent form of sclerema occurring in infants is incontestably the oedematous induration of the cellular tissue. Many of the most recent German writers in the field of paediatrics have described, under the name of induration of the cellular tissue, no other form whatever than sclerema oedematosum. From the anatomical side, this sclerema is characterized by the following phenomena:

When one makes an incision through the skin and subcutaneous cellular tissue into the most indurated parts—e.g. into the calf, the posterior part of the thigh, the glutei—the tissues appear hyperaemic throughout the entire thickness of the cut; the fine vessels are so injected with blood that one can conveniently study their course and position. In consequence, not only the corium and the cellular fat-tissue of the skin appear of reddish coloration, but the fat itself at times takes on a yellowish-red hue. The blood filling the vessels is usually dark and thin-fluid, wells out therefore drop by drop from the mouths of the divided vessels, and even flows off in small quantity from the wound, which is especially well perceived at the cutaneous incision itself. Simultaneously with this, the ground of the incised wound corresponding to the cellular and fatty tissue begins to fill with a yellowish, sticky, somewhat thick fluid which exudes from these tissues. The longer the time elapsed since the incision was made, the more this fluid collects, until at last—after it has filled the whole space of the incised wound—it flows out onto the dissecting table and, mingled with the blood, takes on a reddish coloration. Its quantity is so considerable that from a single incision through the entire thickness of the calf of a newborn child I was able to fill a test-vial to half an inch with this fluid. This fluid infiltrates the subcutaneous cellular tissue, and therefore its interstices appear dilated even to the unarmed eye; in consequence the layer of fat beneath the skin also presents itself not as a firmly coherent, dense mass, but rather as separated—or, better said, divided into small globules, whereby it gains a granular appearance. If one carries the incision deeper, through the muscles, then the interstitial cellular tissue of the latter appears saturated with the same fluid, which likewise flows out of the wound just as it did from the subcutaneous cellular tissue. After the free outflow of the fluid has ceased and one has, by applied pressure, caused the remainder of it to flow off, the skin and subcutaneous cellular tissue again become completely soft and all their hardness disappears.¹

¹ According to Billard, the sclerematous parts become soft merely through the simple outflow of the fluid, without one’s having need to express it.

These are the anatomical characters which I have observed in the majority of cases of oedematous sclerema, and I hold that they may be assumed as the norm for it. In comparison with the descriptions of other authors, the following divergences present themselves:

Valleix and Vogel maintain that in oedematous sclerema the cellular tissue over the aponeuroses transforms into a gelatinous mass of 2 to 4 lines in thickness. According to the assurance of the former, this mass consists of a lamellar tissue greatly distended by accumulated serous fluid, from which the fluid may very easily be expressed. This phenomenon, however, is by no means constant, and I have observed it chiefly in those cases where the subcutaneous cellular tissue was poor in fat, so that this mass in a manner replaced the fat. That the serous fluid may then easily be expressed from it is perfectly correct.

Vogel maintains that in the intermuscular cellular tissue no oedema is ever found in sclerema. This statement is erroneous. On the contrary, I have not once seen that, in a case of oedema of the subcutaneous cellular tissue, it was absent from the interstitial cellular tissue of the muscles.

Bednař says that the subcutaneous fat appears condensed into a yellow- or brown-red, granular mass; that the serum with which the cellular tissue is infiltrated coagulates rapidly in the air; that the vessels of the skin are difficult to inject with a fluid, and that the venous system is usually gorged with liquid blood. The subcutaneous fat does indeed at times appear of yellowish-red colour, but that it is transformed into a brown-red mass I have not observed. The fluid infiltrated in the cellular tissue does at times coagulate in the air, yet this is by no means a constant phenomenon. That the venous system is gorged with liquid blood is perfectly correct; but as regards the slight permeability of the cutaneous vessels, this assertion requires correction—to which we shall, moreover, return later.

Hennig says that the subcutaneous fat appears shrunken together, dark, into a granular mass, and that the lymphatic vessels at times contain a yellow-green fluid. The word “dark” gives no clear, definite concept, for all possible colours can be darker and lighter. If Hennig understands by the expression “dark” a dark-yellow coloration, then such a colour is indeed at times proper to the fat. As regards the filling of the lymphatic vessels with a yellow-green fluid: there is doubtless always lymph contained in the lymphatic vessels, but a greenish fluid I have never perceived in them.

West states that if, while life yet persists, one makes a puncture into the indurated parts, a small quantity of reddish serum flows out from the puncture-wound. This experiment I have never undertaken and can therefore say nothing about it; but I wish, on this occasion, to direct to this means of investigation the attention of those physicians who fear to undertake such a small wound for diagnostic purposes in the simple sclerema of adults.

The question now arises: through what are the anatomical changes of the sclerematous parts conditioned, in the form of induration of the cellular tissue here considered by us? In this respect two chief opinions prevail. Some writers reckon oedematous sclerema to the class of inflammations; others—especially the French—place it in the category of oedemas. With this latter opinion most German paediatricians of the present day also concur. At the time of the dominance of the Vienna School, the inflammatory theory could lay claim to infallibility. Rokitansky, in the first edition of his pathological anatomy, declared all theories which were advanced for the explanation of the essence of oedematous induration of the cellular tissue, and which did not speak for the inflammatory character, to be downright false. Engel and Mildner followed him (Prager Vierteljahrschrift 1847, Vol. II). In time the writers returned again to the old theory of oedema, and this remained dominant so long until Virchow, in his doctrine of tumours, ranged the induration of the cellular tissue of the newborn among the forms of elephantiasis, which, as is known, have an inflammatory origin. Since that time some more recent paediatricians too have begun to count oedematous sclerema among the inflammatory forms. After Hennig (Textbook of the Diseases of the Child, 3rd ed. 1864), who reckons the sclerema among the passive, asthenic inflammations, had expressed his opinion on this subject not quite decidedly, Herzfeld (Diseases of Childhood, 1869) declared outright that the form here considered by us belongs, from the anatomical side, to the category of inflammatory infiltrations of the subcutaneous cellular tissue and of the skin. Let us see, then, what ground such an assertion has:

The hyperaemia of the skin and subcutaneous cellular tissue which accompanies oedematous sclerema could be held to be inflammatory only if it were of active nature and presented, from the clinical as from the anatomical side, all the phenomena which usually appear in the train of inflammation. This hyperaemia, however, has on the contrary a passive, one may even say mechanical, character, while the unequivocal phenomena of an inflammation are completely lacking in oedematous sclerema. Leaving aside the symptomatology of the disease, of which we shall speak further below, let us here fix our eye only on the anatomical picture. It is known that inflamed cellular tissue (connective tissue) is over-rich in cells, which on the one hand are shaped globularly, and on the other hand present the familiar phenomena of division (Theilung). All this taken together yields the picture of so-called inflammatory infiltration. Further, these cells can either be washed away by the fluid oozing out of the vessels—and, together with this, form the fluid known under the name of pus—or else be retained in the intercellular (ground-) substance, transforming, between its compartments, into flat and spindle-shaped structures. In this latter case the inflammation assumes the type of an organizing process, which is characterized by the proliferation of the fibre-bundles of the connective tissue, which exert a pressure upon the other elements and thereby condition the atrophy of the latter. This picture will already be familiar to our readers from the descriptions of the anatomical phenomena of that sclerema which is observed in the non-newborn.

If we now hold these findings against the oedematous sclerema of the newborn, we shall see that the histological phenomena in the latter present nothing similar. That mass of cells which characterizes inflammation is here found neither in the skin nor in the subcutaneous cellular tissue; the fibre-bundles of the connective tissue are by no means increased, and the remaining elements give no sign of atrophy; the fat-globules of the cellular fat-layer, which in the proliferation of the fibre-bundles are given over pre-eminently to this process, preserve their integrity in the oedematous sclerema of the newborn and have a perfectly normal appearance.

Moreover, it cannot be denied that in oedematous sclerema some cells are actually in the state of division—which may well have given Hennig occasion to speak, in describing the anatomical phenomena of the same, of a proliferation of the connective tissue, and which may have induced him to reckon this disease-form to the class of inflammations. Here, however, it must be remarked that the young cells have preserved only their primitive type, and that such a division of them may be observed in newborns who suffer neither from sclerema nor from any other disease of the skin or subcutaneous cellular tissue. This is also quite natural, since the infant organism finds itself in growth and there is no cause whatever why the connective tissue should not grow, when all the remaining tissues are engaged in growth. Let us recall that Virchow, already in his Cellular Pathology, spoke of the resemblance of the morbid phenomena of irritation to the process of growth. On the other hand, in newborns who suffer from oedematous sclerema, that degree of irritation of the skin cannot be denied which is perceived in all newborns in consequence of the new and so unaccustomed stimulus of the atmospheric air, which—both through its chemical properties and through its temperature—becomes a not inconsiderable stimulus. Finally, the oedema in the sclerema of the newborn is an indubitable fact, while the division of the cells of the connective tissue also occurs in that oedema of adults which arises after occlusion of veins, hence from a mechanical cause.

But even if we wished to ascribe an inflammatory character to this division of the cells of the connective tissue, we still could not do so, because according to the most recent investigations the cells of the inflammatory infiltrate are white blood-corpuscles that have emerged from the walls of the vessels. These corpuscles possess the faculty of translocation just as do the corpuscles of the connective tissue, which, according to the observations of Recklinghausen and Hoffmann, take a special part in the formation of pus. When one considers what a mass of serous transudate infiltrates the subcutaneous cellular tissue in oedematous sclerema, it is incomprehensible why this transudate contains no cellular elements and—as Vogel remarks—presents the properties of an ordinary hydropic serum. This almost total absence of cellular elements in the fluid of oedematous sclerema I can fully confirm; and if now and then a white or red blood-corpuscle appears in it, this can be ascribed to the fact that, in the cutting-through of the skin and subcutaneous cellular tissue, vessels situated in a state of considerable hyperaemia are also divided, whereupon the escape of blood from them cannot be surprising.

Herzfeld, however, places oedematous sclerema in the category of inflammations not, say, on the ground of the presence of an ordinary inflammatory infiltrate in it, but rather on the ground that the cellular tissue appears reddish, swollen, and infiltrated with a serous or thick, gelatinous, yellowish, or brownish fluid. As regards the purulent or ichorous infiltration, he says that this rarely occurs. Let us now see how far the serous or gelatinous infiltration corresponds to inflammatory phenomena.

A purely serous infiltration constitutes, as is known, not the essence of inflammation, but the essence of oedema. In inflammation too, oedema does indeed occur, but not in the inflamed parts, rather in the surrounding parts, and shows entirely and completely the character of collateral oedema, which depends on the impeded circulation, or, more simply put, on the blood-stasis in the region of a certain area. Since a truly inflammatory infiltrate is not present in oedematous sclerema, it is comprehensible that the oedema in this disease is not, say, only an accompanying phenomenon, but rather an essential one, and constitutes the essence of the anatomical change in the skin and the subcutaneous cellular tissue. As regards the gelatinous infiltration, Herzfeld takes it for a phenomenon of inflammation and thereby returns to the doctrine of the Vienna School. It is known that Rokitansky maintains that in chronic inflammation a proliferation of the connective tissue occurs which presents itself as a monstrous accumulation of gelatinous masses. This mass consists, in his opinion, partly of gelatinous substance of the connective tissue, reduced from its fibrous substrate, and partly (and chiefly) of gelatinous new-formation of the connective tissue, which is subsequently to become fibrous tissue. A transformation of this kind often leads to adhesions of the connective tissue both with the tissues in which it is enveloped and with the general integuments.

One cannot indeed deny that, in cases of oedematous sclerema that have passed into healing, such gelatinous masses may have been present in the subcutaneous cellular tissue, which—as just mentioned—might have brought about an adhesion of the cellular tissue with the skin and other parts. Yet up to now no such cases are known. Inasmuch as he assumes the cellular tissue degenerated into gelatin to be—at one and the same time—both a product of the disintegration of its fibrous ground-substance and a product of a new-formation, out of which fibrous ground-substance is in turn to develop, Rokitansky evidently falls into a contradiction not to be resolved. In any case the inflammatory new-formation is over-rich in cellular elements, whereas in the gelatinous cellular tissue of oedematous sclerema no superfluity of cells whatever is to be perceived. On the other hand, transformation of the cellular tissue into a gelatinous mass takes place also in simple anasarca, which depends on impeded blood-circulation (Lobstein)—and this, moreover, in adults!

In the newborn, by contrast, one finds, even in the normal state, a copious quantity of gelatinous tissue deposited between the bundles of the connective tissue, similar to that observed in the umbilical cord—inasmuch as at this age the intercellular substance of the cellular tissue, through the action of water, in general yields albumen and a substance having the appearance of mucus. It is therefore comprehensible that under such conditions the transformation of the cellular tissue into gelatinous substance in oedematous sclerema is a very natural process, which by no means requires inflammation in order to find its explanation. Virchow (Doctrine of Tumours, Vol. I, p. 304) ascribes this phenomenon, in his discussion of so-called erysipelas gelatinosum—in which he points out how, at an incision into the affected parts, the subcutaneous cellular tissue of the same appears saturated as with gelatin—to the circumstance that the parts are saturated with much lymphatic fluid. He connects this phenomenon with the cuticula gelatinosa which forms upon the blood emptied in chronic erysipelas, and holds that it approximates to that form of sclerema of the newborn in which, according to the investigations of Chevreul(le), the blood-serum, after the ordinary fibrin has been removed from it, yet coagulates again of its own accord. Thus a disease as decidedly inflammatory as the rose is set over against oedematous sclerema, and the gelatinous infiltrate of the subcutaneous cellular tissue in the one disease as in the other is made dependent on a lymphatic oedema.

The question now arises: are there in oedematous sclerema indubitable signs of a lymphatic oedema, and in what relation to this oedema stands the coagulation of the fibrinogen in the oedematous fluid?

From Virchow’s words one must draw the conclusion that the gelatinous nature of the erysipelatous infiltrate is to be ascribed to the saturation of the subcutaneous cellular tissue with lymph; only it is not quite possible to see whether he ascribes this phenomenon to the chemical action of the lymph upon the cellular tissue, or to the gelatinous constitution of the lymph itself mechanically accumulated in the said tissue. The former supposition is refuted by the fact that in elephantiasis Arabum too a lymphatic oedema takes place, while a gelatinous transformation of the cellular tissue is not perceived in this disease; the latter, by the fact that lymph does not possess a gelatinous consistency. If the gelatinous appearance of the cellular tissue in oedematous sclerema is supposed to depend upon its being saturated with lymph, then in any case the fluid expressed from the oedematous tissue would have to contain the formed elements of lymph—which, however, as we already remarked above, is by no means the case.

One might object to us that Virchow, under the expression “lymphatic fluid,” understands not lymph alone, but also the so-called fibrinogenous fluid, which coagulates in the air into a gelatinous coagulum. This fluid can, in his opinion, form in the interior of the parts themselves, namely as a consequence of an irritative process which conditions the transformation of any other material into fibrinogenous substance. But, in the first place, it is not proven that the fibrinogenous substance effects a gelatinous transformation of the cellular tissue; secondly, the exclusive origination of the latter in consequence of an irritative process is very problematic; thirdly and finally, fibrinogen is contained in every transudate, but its coagulation occurs only in the presence of fibrinoplastic substance.

Far more admissible seems Virchow’s other explanation concerning the presence of lymphatic fluid in the tissues and the coming-about of the lymphatic oedema, which he derives from a stasis of the actual lymph in the lymphatic vessels—as a consequence of the swelling and impermeability of the lymphatic glands. It is comprehensible that when an organ through which the lymph has to take its passage becomes obliterated, this latter will accumulate in the larger and smaller lymphatic vessels lying below the obstruction. But such a swelling and impermeability of the lymphatic glands does not take place in oedematous sclerema—and therefore the supposed lymphatic oedema occurring in this disease cannot be explained in this way.

The coagulation of the fluid infiltrated into the subcutaneous cellular tissue in oedematous sclerema requires, for its explanation, neither an inflammatory theory nor the assumption of a lymphatic oedema, but rather finds, according to the investigations of Al. Schmidt (On the Fibrin-Substance and the Causes of its Coagulation. Arch. für Anat. u. Physiol. 1861), its most correct interpretation in the union of the fibrinoplastic with the fibrinogenous substance. The former constitutes the albuminous part of the content of the red blood-corpuscles, even when the red blood-corpuscles do not participate directly in the coagulation. Among the number of factors of coagulation belong—besides the chyle, the lymph, the pus, etc.—also those fluids into which the content of the cells, and among them that of the blood-serum, has passed. As regards the coagulable substance (the fibrinogenous), it is found in all transudates; and the power of separating out a part of the albuminous substance in a coagulated state is possessed not only by the spontaneously coagulating (blood, lymph, pus), but by almost all serous fluids. They coagulate through the accession of blood or another fibrinoplastic fluid—and often they fail to coagulate only because the factor of coagulation is lacking to them. Where the fibrinoplastic substance is present in small quantity, there the coagulum itself gains the appearance of a gelatinous precipitate. Among other transudates, Schmidt has also investigated the transudate of oedematous sclerema and found that it fully obeys the general laws of coagulation.

It is known that transudates which one allows to stand in the air can coagulate of themselves, which probably depends on the fact that the blood-serum itself, as already said, serves as a factor of coagulation. Besides this condition, which can bring about a coagulation of the oedematous fluid of sclerema, it must not be left out of account that, at an incision, drops of blood may easily be admixed to the oedematous fluid from the gorged vessels of the skin and subcutaneous cellular tissue, which straightway supplies to the transudate fibrinoplastic substance; and it does not here come into consideration that perhaps only a small quantity of blood is admixed, for it is known how Schmidt, after he had merely drawn a streak of blood over the inner surface of the reaction-vial and had then filled this to the half with transudate from the pericardium, obtained a gelatinous coagulum. On the other hand, the non-coagulability of the transudate is explained from the same cause. If the blood-serum contains no fibrinoplastic substance, and the incision is made in such a way that no drop of blood can mingle with the transudate—then no coagulum arises.

Although there are no sure signs of the indubitable presence of a lymphatic infiltration in oedematous sclerema, yet a certain degree of stagnation in the lymphatic vessels cannot be denied, in consequence of which, at an incision carried through the skin and subcutaneous cellular tissue, lymph may very well pass into the transudate, which in turn furnishes a factor for coagulation. According to the investigations of Ludwig and Tomsa, the quantity of lymph in the lymphatic vessels and lacunae increases with heightened blood-pressure in the veins, in those oedemas which arise in consequence of an obstruction of the circulation in the veins. All this takes place more or less in oedematous sclerema, and from this it is explained how one at times comes to perceive dilated lymphatic lacunae under the microscope. But Tomsa’s own investigations speak decidedly against the inflammatory nature of the lymphatic oedema, because the content of the lymph-lacunae in inflammation is very different from the ordinary lymph. In parenchymatous inflammation of the connective tissue, the lymph-lacunae play only a passive role and merely take up the products of the formative changes of the connective-tissue bundles and their corpuscles.

From what has been said, it emerges sufficiently that one may not attach to the oedematous fluid of the sclerema of the newborn the significance which Chevreul(le) and Virchow have striven to attach to it, and that it has nothing specific about it. As is known, the former assumed that the hardness of the parts in oedematous sclerema depends on the peculiar constitution of the serum infiltrating the connective tissue and on its coagulation in the subcutaneous cells. The latter ascribed this hardness to a lymphatic oedema, which—in his opinion—differs from the ordinary in that the affected parts do not present that doughy consistency which readily yields to external pressure, but rather appear hard and taut and offer a greater resistance to the pressure of the finger. But when one takes into consideration that this sclerematous hardness is also observed in those oedemas which depend on an obstruction to the return of the venous blood—as, for example, in organic heart-defects, where the oedema is indubitably of serous constitution—then it becomes clear that, as regards the production of the hardness in the affected parts, the chief role is played not so much by the nature of the accumulated fluid as rather by its quantity, and that the hardness is conditioned especially by the distension and the not-further-yielding compliancy of the parts.

On the other hand, the seat which the oedema occupies exerts no small influence upon the softness or hardness of the swollen parts. If it occupies only the subcutaneous cellular tissue, then the skin, as a yielding and distensible tissue, will, at an inconsiderable degree of the oedema, not attain to the degree of a taut tension; but if the oedema has its seat in a cellular tissue covered by aponeuroses, then these, as unyielding structures, will soon be distended to the degree of the tautest tension.

Of a coagulation of the sclerematous fluid in the cellular tissue there can—according to Virchow’s opinion—be no question at all, because a fibrinogenous fluid is subject to coagulation only in the air. According to Schmidt’s investigations, however, oxygen and the atmospheric air have no direct influence upon coagulation; on the other hand, according to the experiments of the same investigator, the blood and the transudates coagulate—albeit slowly—even in an air-void space. If, however, coagulation does not occur, this happens only because from an enclosed space the accumulated carbonic acid cannot escape, which of itself most decidedly retards coagulation. If one now considers that oedematous sclerema frequently coincides with atelectasis of the lungs, as in general with an incomplete oxidation of the blood, then from this emerges the chief condition which impedes the coagulation of the sclerematous fluid in the cellular tissue.

Although the assumption of a specificity or non-specificity of the sclerematous fluid could receive its final decision only after the publication of Al. Schmidt’s works, there was even before this no lack of facts contradicting Chevreul(le)’s opinion. Thus, for example, Billard, to test this opinion, subjected to the influence of the air the serum from the oedema of the subcutaneous cellular tissue of a two-year-old child that had died of gastro-enteritis, and another serum from the cellular-tissue oedema of an adult who had died of heart-disease—and the one serum as well as the other coagulated. Likewise coagulated the serum which he had taken from the cellular tissue of healthy children. Relying on these facts, Billard contested the correctness of Chevreul(le)’s view, and his experiments would have lost nothing of their value had not Bouchut repeated them and found in doing so that the serum taken from the cellular tissue of a child suffering from anasarca coagulated in the air. At the present time such an experiment would have no purpose whatever and could in no case cast doubt on the correctness of the Billardian experiments; for why transudates coagulate at one time and not at another remains, after Schmidt’s works, no longer a riddle.

Is anything still to be said about the coagulability of the blood-serum from which the ordinary fibrin has been removed, adduced by Virchow in favour of the specificity of sclerematous oedema? Schmidt removed the coagula repeatedly from the spontaneously coagulating serum taken from a corpse, and it continued to coagulate again and again. When at last it ceased to coagulate, one could, by the admixture of blood, again obtain a fresh coagulum.

And thus we must, on account of the absence of unequivocal signs of inflammation, adopt the opinion of those writers who regard the sclerema of the newborn as an oedema. That an oedema is present is denied even by those who are convinced of the inflammatory nature of the sclerema. The whole difference consists only in this: that the one party regard the oedema as a consecutive phenomenon of inflammation, dependent on the impermeability of the local vessels, while the others see in the oedema the chief phenomenon underlying all the local anatomical changes.

All that we have said hitherto refers, however, only to the pure oedematous sclerema, whereas there are without doubt cases where the oedema—as we have just said—is only the local accompaniment of an inflammatory infiltrate; indeed, where the oedematous sclerema appears as the accompaniment of a general disease which finds its expression in local inflammatory phenomena. It is very probable that cases of this kind gave precisely the occasion to reckon oedematous sclerema to the class of inflammations. In confirmation of what has just been said, we permit ourselves to adduce the following case:

On the 21st of September there was admitted into one of our infirmary wards a girl a month and a half old, of weak bodily constitution (7 pounds in weight, 19 inches in length), with an incipient erysipelatous process on the right hip. The affected place was somewhat swollen, of pale rose-red coloration and sensitive to pressure; the consistency of the swelling was not hard, its temperature, like that of the whole body, not considerably elevated. With the exception of restlessness and sleeplessness, no other disturbance of the functions was to be perceived in the patient. On the 22nd and 23rd the swelling spread farther on the right thigh, and the redness took on a darker shade. On the 24th, considerable fever-heat set in in the child; swelling and redness had spread on the right extremity over the knee down to the foot. The swelling appeared fairly hard, hot, and painful, so that the disease had already assumed the character of a phlegmon. At the same time swelling and redness also appeared at the left knee. On the 25th the swelling had taken possession of both extremities, the redness everywhere—with the exception of the inner side of the right foot—having disappeared. The affected parts were of hard consistency, and the swelling had altogether assumed the character of a sclerema, while the heat of the body somewhat diminished. On the 26th and 27th the temperature fell still more (37.7° C.), and the swollen extremities (excepting the right foot) appeared pale and very hard; yet swelling and hardness were not uniform, and here and there, especially on the thighs, softer depressions and transverse stripes were perceived, so that the extremities had a somewhat knotty appearance; the skin, however, could nowhere be raised into a fold. On the 29th the swelling distributed itself on the feet—its character remaining the same—more uniformly. The knees (especially the popliteal hollows), which had hitherto remained almost free of the swelling, now also swelled up strongly and appeared likewise of white coloration. On the 1st of October the fever-heat again increased, the swelling on the feet somewhat decreased, but on the other hand the upper extremities too swelled. Here the swollen parts were perfectly pale and hard, as in sclerema; the swelling, however, was not uniform, presenting here and there depressions; the wrist-joints had remained almost free. On the 2nd the swelling of the hands had increased and had become more uniform, but showed at the same time also a greater hardness. On the 3rd the fever-heat decreased. On the 5th the swelling on the hands became still stronger, while on the feet it decreased; the pale redness of the right foot persisted. On the 6th a uniform, somewhat doughy swelling appeared on the hairy part of the head, while the colour of the general covering of the skull remained unchanged, but the fever-heat increased considerably. The strength of the child sank, and it ceased to take the breast. On the 7th the swollen parts remained in the same state, but the weakness increased; on the 8th the child died.

At the autopsy the swollen and indurated places proved to be strongly oedematous, the oedematous transudate at first sight in no way differing from the transudate of true oedematous sclerema—that is, the serum infiltrating the subcutaneous cellular tissue was just as yellowish, sticky, and thick as in oedematous sclerema. Noteworthy, however, is the fact that the oedematous parts appeared rather anaemic than hyperaemic; the oedematous subcutaneous cellular tissue of the head-coverings presented perfectly the appearance of a gelatinous infiltration, while at the right foot, where the redness had persisted through the whole duration of the disease, an actual purulent focus (abscess) was found. The serum of the oedematous parts examined under the microscope did not show the characters of pus, although far more formed elements (white, and altered, shrunken-together red blood-corpuscles) were contained in it than in the serum of a simple oedematous sclerema. On the other hand, the oedematous cellular tissue was over-rich in cells, which were deposited in it as round lymphoid elements, whereby the ground-substance itself appeared exceedingly pale and almost translucent, with only indicated, half-effaced fibres. As other changes there were found in the corpse: oedema of the pia mater and of the brain, a bronchopneumonia duplex, and a hepar adiposum.

We have adduced this case intentionally in order to show what difference exists in the histological phenomena between the truly oedematous and the inflammatory-oedematous sclerema, notwithstanding the apparent resemblance of the microscopic-anatomical phenomena of the two. In clinical respect too there exists no inconsiderable difference. In the case adduced by us, the oedema did not strictly follow the laws of gravity, as is wont to be the case in truly oedematous sclerema, and the temperature did not sink so low as in the latter, but kept always at a certain height—indeed, rose more than once above it.

As is known, oedemas very frequently accompany the puerperal processes of the newborn, processes which are prone to spread over extensive territories of the body. When such an oedema pitches its seat in the subcutaneous cellular tissue, then it can present the same phenomena of induration as does oedematous sclerema; only in this case there exists, together with the oedema, an inflammatory infiltrate of the cellular tissue, which allows a greater or lesser quantity of lymphoid elements (pus-corpuscles) to be perceived within it. The oedematous fluid itself is not a simple transudate of blood-serum, but a transudate very rich in formed elements, which former in turn are a product of inflammation. According to Buhl’s opinion, the familiar form of pyaemia consists, both in the mother and in the newborn, in an inflammation of the lymphatic vessels, which in consequence lose the faculty of absorbing again the blood-serum exuding from the vessels into the tissues, on which the oedema of the tissues precisely depends. The proliferating cells, however, in the inflammatory puerperal processes undergo a rapid destruction, decomposition, and dissolution, in consequence of which the transudate absorbed by the blood-vessels in the affected part is now carried on into all possible parts of the body, where, escaping together with the blood-serum into the healthy organs and tissues, it calls forth the secondary inflammations of the lymphatic vessels and the inflammatory infiltration of the connective tissue.

But whatever form of the puerperal process of the newborn we might have to do with, we can—even after the characteristic changes of the internal organs which are observed in these processes—yet never confuse them with the simple oedematous sclerema. (See Hecker and Buhl, Klinik der Geburtskunde, Vol. I, pp. 255–288.) It seems to me, however, that the oedematous sclerema observed in the puerperal processes of the newborn is not always conditioned by an inflammatory process of the subcutaneous cellular tissue; I have at least observed cases where the sclerema developed according to the same laws of gravity as does the simple oedematous sclerema, while in the internal organs there were nevertheless found such changes as indicated a puerperal process of the newborn. There were found in the oedematous cellular tissue of the lower extremities no signs of an inflammatory infiltrate. A priori such a phenomenon is easily explained: in the liver and the kidneys we here find such alterations as—according to Buhl’s words—point to a parenchymatous inflammation; but it is known that such an inflammation of the kidneys alone already suffices to call forth an oedema of the subcutaneous cellular tissue. What is furthermore of still greater importance is that one observes in the puerperal processes of the newborn also changes in the heart, which are characterized by the fact that the transverse striations of its primitive muscle-bundles lose the purity of their contours, appear half-effaced, and allow a granular deposit to be perceived—a circumstance which conditions a weakness of the heart-contractions and a stagnation of the blood in the venous system. The ecchymoses so frequently observed in these processes, under the serous membranes of various organs and in other tissues, point in their turn to a loosened coherence of the vessel-walls, and this is again a circumstance which retards the blood-circulation and promotes the stagnation of the blood.

But even if the oedema—in the anatomical sense—is regarded also as the cause of the familiar local changes, still, in the pathological sense, its independence can by no means be proven. The chief role in the coming-about of the oedema is played by the impeded blood-circulation and perhaps also by a peculiar constitution of the blood itself. What obstructions of the blood-circulation, then, take place in the newborn suffering from oedematous sclerema?

All investigations which had for their purpose the establishment of the anatomical character of these obstructions have led to negative results, and the only thing that one has attained through them is the supposition of an obliteration of the capillaries of the skin. This supposition has developed itself as follows:

Bouchut resolved—after he had examined the organs of the blood-circulation in those who had died of oedematous sclerema and had found all the larger arterial and venous trunks patent—to inject the lower extremity of a corpse in order to investigate the patency also of the capillaries. For this purpose he took turpentine (?) and injected the arteries with it. “In all the children”—he said—”the fluid injected into the artery penetrated into the superficial as well as the more deeply lying capillaries, coloured skin and muscles, and passed over into the veins; but even when the injection-mass passed over also into the veins, this occurred, as regards the extremity of the said corpse, only with difficulty. Instead of penetrating all the capillaries, the injection-mass filled only a small number of the capillaries of the skin and of the subcutaneous cellular tissue, while it completely penetrated the capillaries of the more deeply lying organs and coloured the muscles.” We believe we must draw attention to the fact that Bouchut himself attached no great value to his experiment, in expectation of whether later investigations might perhaps confirm his obtained results.

Hennig (Schmidt’s Jahrbücher 1862, No. 2) repeated Bouchut’s experiment and arrived at a different result. He injected a carmine-containing gelatin solution and found that the injection-mass, although it could be injected only with some difficulty, yet coloured the skin as well as the muscles of the extremity; only the colouring of the skin covering the Achilles tendon occurred less completely. The untenability of Bouchut’s supposition can also be demonstrated a priori: all authors agree in this, that the veins of the child suffering from oedematous sclerema are overfilled with blood. Could this take place if the capillaries of tissues so widely distributed, such as the skin and subcutaneous cellular tissue, were impassable? Since he did not find Bouchut’s experiment confirmed, Hennig came to the conclusion that the ground of the disturbed circulation is to be sought in a paralysis of the cutaneous vessels, and hence in an altered relation of the capillaries to the corresponding cellular territories.¹ This role imposed upon the cells indicates that Hennig believed he saw in the paralysis of the cutaneous vessels a peripheral paralysis of the vasomotor nerves; but the oedemas in sclerema occur not only in the subcutaneous cellular tissue, but also in the cellular tissue of internal parts, as, e.g., of the pia mater; further, together with such oedemas there exist at the same time also hydropic accumulations in the thoracic and abdominal cavities. If, then, one is to speak of a paralysis of the vessels, then not only the cutaneous vessels, but rather the vessels of such widely extended territories, must be comprised thereunder—so that a peripheral origin of such a paralysis is hardly to be assumed. The central origin of so extensive a paralysis of the vessels would, however, be scarcely compatible with the continuance of life.

¹ I have likewise repeated Bouchut’s experiment and took for the injection first a solution of ferrocyanide of potassium and then of sulphate of copper. From the union of the two there forms a precipitate of ferro-sulphocyanide of copper (sic), which coloured the vessels brown-red. I chose this injection-method because the injection-mass, remaining perfectly fluid, penetrates more easily into the fine vessels. The microscopic objects of the skin and of the subcutaneous cellular tissue everywhere showed a uniform injection of all the capillaries.

If one is willing to admit the view of the independent role of the vessels, then it would, in my opinion, be best to adhere to the theory of Rigal (de l’affaiblissement du coeur et des vaisseaux dans les maladies cardiaques. Thèse. Paris 1866), according to which the hyperaemias and oedemas depend on a weakness of the capillaries, which can exist together with a weakness of the heart, or also of itself alone. It is comprehensible that in the latter case the heart must perform more vigorous contractions in order to overcome the obstruction set to the circulation on the part of the capillaries. If, on the other hand, the weakness of the capillaries coincides with that of the heart, the contractions of the latter become weak and scarcely perceptible, the heart-sounds indistinct, the pulse weak, intermittent, and irregular. The causes which underlie the weakness of the heart and of the vessels are, according to Rigal’s opinion: diminished nerve-influence, organic changes, and stagnations occasioned by a mechanical obstruction of the blood-flow.

We are far from ascribing infallibility to Rigal’s theory; we could, on the contrary, ourselves raise several objections against it, but this does not lie within the plan of our present work. As regards its relation to the theory of oedematous sclerema, the phenomena of induration of the cellular tissue are better explained by the Rigalian than by the Hennigian theory; for the oedema underlying it is in fact conditioned by weakness of the organs of the blood-flow, whence arises stagnation of the blood in the vessels of the skin and subcutaneous cellular tissue, just as blood-stases in other organs have as their consequence internal oedemas and hydropic exudations upon the free surfaces of internal cavities.

Vogel explains the origin of the sclerema from an insufficient innervation of the heart-muscle, in consequence of which the latter contracts more seldom and calls forth both a sinking of the temperature of the whole body and a peripheral transudation; in other words: Vogel wishes to derive the oedema underlying the sclerema from a weakened heart-function. The oedema, like some other phenomena, does indeed find its explanation in this; but have we a right to ascribe the sclerema to the weakened function of any single organ, when a general weakness underlies the disease, expressing itself through a weakened function of all the organs? The nervous system doubtless plays an important role in the normal life of the organism, but one cannot ascribe everything to it either, since its activity is dependent on the regular function of the organs of the blood-movement and respiration.

One need only observe a child afflicted with oedematous sclerema in order to convince oneself of the general weakness present. The patients are mostly non-carried-to-term and not fully developed children, in whom a sufficient energy of the organs is not at all to be expected. Under all circumstances they are small, and this smallness depends precisely on the incompleted development of the soft as of the firm parts of the body; after they are swaddled, they sleep almost incessantly; when one loosens the swaddling-clothes, they move their extremities, on awaking, only slowly, and instead of crying give mostly only a whimper of themselves; a loud, sustained crying one never hears from them, and when they do cry once, it happens only with a weak voice. Their respiration is superficial and retarded, indeed is momentarily quite interrupted; their thorax expands only with difficulty, the contraction of the abdominal muscles is scarcely perceptible. In consequence of so incomplete a respiration, the blood is not properly oxidized and takes on a venous character, whence the parts in which the blood stagnates take on a bluish coloration with various shades. The weakness of the respiration conditions also a sinking of the body-temperature to 33° C. and below. The contractions of the heart are retarded, weak, the second sound scarcely audible; the pulse is small, slow, makes at most 60–70 beats in the minute. In consequence of the weakened heart-movement, the blood stagnates in the capillaries and veins, whereby passive exudations of the blood-serum into the parenchyma of the tissues and upon the free surfaces of the internal cavities are conditioned. The sensibility is in general blunted, the need for nourishment slight; the necessary strength for sucking is also lacking, so that the child lets go again the breast it has seized; even swallowing is difficult, and the peristaltic movement of the bowels so slight that the meconium is for a long time not evacuated; the urine-secretion too proceeds slowly and sparingly, etc.¹

¹ The phenomena adduced here as proof of the general weakness refer, however, only to the initial period of the disease; in the sequel, when oedemas have already formed, all the symptoms gain in intensity, for the weakness of the functions passes over into a suppression of them: thus the drowsiness may heighten to lethargy, the temperature sink to 22° C., the movement be still more impeded in consequence of the induration of the intermuscular cellular tissue, etc.

The course or spread of the sclerema testifies quite especially to the weakness of the blood-movement organs; the oedemas follow the law of gravity and indicate how the force of the heart no longer suffices to give the blood-mass the necessary movement. The induration begins from the feet, spreads over the nates, the back, the genitals, the mons veneris, the belly, and only thereafter over the upper extremities and the face. The same course is taken also—according to the observations of Rummel—by the high-red coloration of the skin.² The oedemas thus form everywhere in connection with the stagnation of the blood, and this appears in those regions of the periphery upon which the influence of the heart-force already begins to fail. The more this force weakens, the more the limits of its efficacy narrow toward the periphery, until the heart is at last no longer able to drive the blood even into the organs lying nearest to it. But not only there—also in every peripheral part the oedema follows in turn the laws of gravity, and behaves like a hypostasis. Although the foot lies farther out than the knee, the sclerema, since the child lies constantly on its back, yet appears first in the calf and only then on the foot, because the position of the latter is higher than that of the calf, and the heel possesses a less loose cellular tissue. On the posterior part of the trunk the sclerema likewise appears earlier in consequence of the recumbent position; but if one gives the child a lateral position, then the sclerema too appears first on the side in question.

² According to my observations, this coloration is rather bluish-red, dependent on the venosity of the stagnating blood. Red is the skin in the newborn always, in consequence of the stimulus of the air, which acts upon it as a new stimulus. Perhaps, however, a mechanical irritation, to which the children are exposed through the washing immediately after birth, has a part in this too. This redness passes in most cases, after some days, into a yellowish coloration. In children not carried to term, this redness is wont to be especially intense, and that disease-form which one calls erythrosis to be pre-eminently observed in them. The difference of the coloration of the skin in sclerema depends especially on this cause: so long as only blood-stagnation is present, the skin appears more or less bluishly coloured; then, when oedema has already occurred and the capillaries have become free, the change of coloration follows the same laws as in erythrosis, i.e. the reddened skin becomes yellow and then pale. Above I mentioned that in sclerema an irritation of the skin takes place to the same degree as in newborns generally, and this I refer especially to that degree of irritation which conditions the erythrosis, and to the causes from which this arises.

One must not, however, leave out of account that the blood-stagnations in the venous system are dependent on the degree of the free blood-movement in the lungs and on the unhindered emptying of the right ventricle occasioned thereby. The free blood-movement in the lungs goes hand in hand with an unhindered and full respiration; in children suffering from sclerema the respiration is exceedingly weak, superficial, and retarded, and the lungs, which do not properly expand, therefore very frequently present atelectasis in some of their provinces. This is so important a causal relation that many writers bring the sclerema into connection exclusively with an impeded respiration—indeed, some identify it with asphyxia (as, e.g., Letourneau. Quelques observations sur les nouveau-nés. Paris 1858).

When we fix our eye on the pure, congenital atelectasis of the lungs, we have in this all the conditions for the origination of oedematous sclerema, since, with impeded circulation in the lungs, a stagnation of the blood in the venous system must here occur. Further, there exist here the common symptoms of both congenital atelectasis and of sclerema: weak and superficial respiration, weak and retarded heart-beat, sunken temperature, etc. And yet no sclerema is present. This circumstance is the more surprising, since in acquired atelectasis of the lungs—in consequence of an early-acquired, or even congenital, catarrh of the bronchi—oedemas and serous exudations appear in the cavities (Hennig). The solution of this riddle lies in the fact that here the transudates appear only after an eccentric hypertrophy of the right heart, which does not take place in congenital atelectasis. It is therefore the disturbed lung-function that is of itself not yet sufficient for the production of the sclerema, and the derivation of this disease from the various affections of the respiratory organs remains in any case problematic.

But there is a circumstance in the atelectasis of the lungs capable of explaining the absence of oedemas in yet another way, namely this: that here—notwithstanding the restricted permeability of the lungs and the weakened heart-action, hence notwithstanding all the conditions for a stagnation of the blood in the veins and for a venous constitution of the blood—the skin of the patients yet shows no bluish coloration, and cyanotic phenomena appear only temporarily, namely only during a convulsive contraction of the glottis, hence in such a moment as that in which the stagnation in the venous system and the deficiency of oxidation of the blood reach their highest degree. This circumstance can serve as proof that the stagnations of the blood in atelectasis are not so considerable as to condition serous transudates.

If we further turn to those disease-forms which have a general weakness as their basis, and which through a definite series of phenomena approach the sclerema, then we necessarily strike upon that form which Hervieux has described under the name of algiditas progressiva neonatorum primitiva. This form is characterized not only by a progressive sinking of the body-warmth, but also by a simultaneous impairment (depression) of the blood-movement and respiration; it also approximates to sclerema in that the movements of the patients, like their voices, are weak, the sensibility appears blunted, etc.; nevertheless there is—despite the blue coloration and turgescence of the extremities—no actual sclerema present, and those suffering from this algidity have a pale, colourless appearance and not seldom find themselves in a state of marasmus, so that they look like childish greybeards.

Löschner, who denies the independence of this disease and regards the sclerema only as a stage of it, has nevertheless had to admit that the first stage of the algiditas progressiva can exist without oedema and discoloration of the cellular tissue, and that the latter is observed only in an anomaly of the vessels and considerable stases in the capillaries. Hervieux, however—who, as is known, bases his conclusions concerning this disease on the physiological experiments of Milne-Edwards and Chossat, approximates it to the sclerema, and very well knows that it often appears as a sequela after other diseases—has, notwithstanding, assumed the primitive form to be an independent one and has sought its distinguishing mark in the fact that it exists without sclerema. Guillot—the referee of the commission to whose judgment the Société médicale des hôpitaux de Paris had handed over Hervieux’s investigations—finds the starting-point of the decline of the temperature, as of all the other phenomena, in the weakness of the respiration, with which one may of course concur.

Let us now pass over to congenital debility of life (angeborene Lebensschwäche), and if we follow the description which Bednar gives of it (Textbook of Children’s Diseases, 1856, p. 579), then we strike upon the following phenomena: small (11–15 inches) body-length, redness and coldness of the skin, great density of the subcutaneous cellular tissue, which is filled with a yellowish, granular fat or with a yellowish, tough serum; predominant sleep; instead of the full voice only a whimpering; in sucking a feeble grasping of the nipple; slow swallowing, so that in these children not seldom choking-fits occur; incomplete respiration, retarded heart-beat, sunken belly, empty (owing to absence of the testicles) scrotum, bent posture of the body, delayed evacuation of the meconium, and a late falling-off of the umbilical-cord remnant. In the corpses of the children the blood is wont to be dark and fluid, at times resembling meat-water, at times, however, also thick, always without fibrinous coagula, only seldom with loose coagula in the heart-cavities. The various organs present now hyperaemia, now anaemia, now oedema. This debility of life occurs mostly in non-carried-to-term children; if one finds it in those carried to term, then these are usually twins, triplets, or such as, in consequence of diseases of the mother or of the fruit, are born weak and not fully developed. Who will not find again in this description all the symptoms of oedematous sclerema, and must one not wonder that Bednar found it necessary, in the same work, to adduce the sclerema as a special disease-form?

One cannot, however, refrain from perceiving that Bednar, with regard to the non-carried-to-term children who, in consequence of their defective development, eo ipso must suffer from debility of life, yet in much diverges from those writers who—although they admit the red-bluish coloration of the skin—yet maintain that the non-carried-to-term children have a wrinkled, senile face and dry, wizened extremities, which, as is well known, cannot be the case when the subcutaneous cellular tissue is permeated with fat and fluid. Some of these writers even maintain that, although the corpses of the non-carried-to-term children show a bluish or blue-red coloration of the skin, the limbs yet preserve a more or less great flaccidity or flexibility, which in their opinion depends on the absence of rigor mortis (Mende).

I, for my part, must say that, with regard to the non-carried-to-term children, both Bednar and the writers who contradict him are right only up to a certain degree. The wrinkled face and the wizened extremities do indeed occur in non-carried-to-term, seven-months children; but in the majority of the eight-months or still later born non-carried-to-term children, face and extremities always show a considerable fullness, and the children really bear many of those peculiarities which Bednar ascribes to them, with the exception of the everywhere and always asserted heightened density of the subcutaneous cellular tissue. The same holds also of twin-children.

Relying on these facts, I believe I must maintain that the subcutaneous cellular tissue develops especially between the 7th and 9th month of pregnancy, since children carried to term—with only few exceptions—are always distinguished by a considerable fullness and roundness of forms, which depend on the aforementioned development of the cellular tissue and the copious fat-deposition into it. This again explains why, in children suffering from oedematous sclerema, the cells of the connective tissue find themselves in a state of division, and why this disease has the appearance of an irritative process, which so easily leads to the conclusion of an inflammatory nature of the sclerema.

It is comprehensible that, together with the connective tissue, its vessels also develop. This, however, can be achieved only with a copious nourishment of these parts, i.e. with a copious inflow of the blood to them—and herein precisely lies the cause of the hyperaemia of the subcutaneous cellular tissue, which in its turn constitutes one of the causes of oedematous sclerema.

But even if Bednar goes too far with his assertion that the subcutaneous cellular tissue in non-carried-to-term children is always in a state of induration or of oedematous sclerema—yet it does not follow from this that it is never so. On the contrary, this morbid state of the subcutaneous cellular tissue is not seldom observed; only it is not generally distributed, but restricts itself to the lower extremities, especially to the calves; thus, as regards its localization, it follows the same order that oedematous sclerema in general follows, which in the majority of cases begins from the feet and especially the calves. The cause of this phenomenon is, in the one case as in the other, grounded in the fact that the blood, in the weakness of the heart-contractions, stagnates in the extremities and especially in the feet, as the most distant parts. The difference between the non-carried-to-term children and those suffering from oedematous sclerema consists, however, in this: that in the former the oedema of the feet soon passes off again and the lower extremities regain their natural softness, whereas in those suffering from sclerema the oedema increases and passes over to other parts—in other words: in the non-carried-to-term children the heart gains, with the development of the body and its organs progressing after birth, a greater force and contracts more energetically; but in those suffering from sclerema the force of the heart-contractions steadily decreases, and the blood-stagnation in the veins and capillaries continually gains more ground.

In this connection I can adduce the following statistical data: Of 275 non-carried-to-term children whom I observed for this purpose, induration of the calves appeared in 182, while 93 remained free of it. Of the 182 with indurated calves, the induration spread to other parts (i.e. general sclerema formed) in 52, while 34 remained alive—that is, the induration of the calves passed off in them and the sclerema did not spread further. These children developed subsequently and enjoyed good health.

In the remaining 96, the hardness of the calves likewise passed off, and the sclerema did not spread over other parts. They lived after birth a longer or shorter time and died of various diseases which stood in no connection whatever with oedematous sclerema.

In some cases of oedematous sclerema I have made small incisions into the skin at the not-yet-affected places, and never seen any considerable haemorrhage from the divided capillaries, whereas, as is well known, in newborns every wound has a considerable haemorrhage as its consequence; indeed, in some cases the blood even welled forth only scantily from the wound—a phenomenon which likewise testifies to the weakness of the contractions of the heart and of the arteries. It has, moreover, occurred to me to observe, even in well-carried-to-term, just-born children, an induration of the calves, which, however, disappeared in the following days. In some, this disappearance coincided with the general emaciation of the body and could be ascribed to the decrease of the fat too copiously deposited into the subcutaneous cellular tissue during uterine life; in others, however, the softening of the calves did not coincide with the general emaciation, and in this case the hardness of the calves was probably caused by an oedema of the subcutaneous cellular tissue, which in its turn may have been conditioned by a weakness of the contractions of the heart and of the arteries and a heightened looseness of the walls of the capillaries.

Can one trace oedematous sclerema back to a congenital or acquired adynamia of the muscles, as Legroux (considérations sur la nature et le traitement du Sclerème et de l’asphyxie. Gaz. des hôpit. 1857, No. 46) has attempted to do? The adynamia of the muscles announces itself, according to the observations of this investigator, through weakness of the respiratory movements, through weak contractions of the heart and of the arteries, through stagnation of the blood in the capillaries, through oedema, cyanotic coloration of the skin, a steadily increasing cooling of the body, and finally through the impossibility of sucking and swallowing, which also renders all nourishment impossible. The starting-point of the affection is, according to Legroux, an insufficiency of the mechanical act of respiration, whence the sclerema is an asphyctic disease. In the heart itself, which participates only in part in the general adynamia of the muscles, the phenomena of asphyxia manifest themselves: the movements of it become, under the influence of this cause, ever weaker and weaker. If the sclerema, on the side of the lungs, is an asphyctic disease, then it is, on the side of the heart, a syncopal one. The arterial blood moves with an insufficient force, does not give the venous blood the impulse required for its onward movement, and therefore it stagnates in the veins. From this very cause proceed the retarded circulation in the capillaries and the oedema.

If one considers that the heart is a muscle, that in the walls of the arteries involuntary muscles are distributed which play so important a role in their contractions, that finally the contraction of voluntary muscles too—e.g. those of the extremities—has a very important influence upon the onward movement of the blood in the veins, then one cannot refuse a certain measure of recognition to Legroux’s conclusions. Notwithstanding, one cannot trace back all the phenomena in sclerema to the adynamia or weakness of the muscles alone. My observations on non-carried-to-term children have shown that among them there occurred such as united in themselves all the conditions for the production of sclerema, in whom it nevertheless did not appear. The mentioned non-carried-to-term children presented all the phenomena of muscle-weakness in the sense in which Legroux conceives it: they breathed weakly, were cold, and—what is especially important—had constantly a bluish skin-coloration, but withal no hardness could be perceived in the skin and in the subcutaneous cellular tissue. The persisting cyanosis proved that the blood, in its incomplete oxidation, stagnated in the lungs and in the capillaries; but oedemas were nevertheless not present. Therefore one is precisely compelled to assume that the muscle-weakness and the stagnation of the blood in the capillaries are not of themselves alone sufficient to call forth an oedema and the induration of the subcutaneous cellular tissue conditioned by it, but that there is further needed a special looseness of the walls of the capillaries in order that the oedematous transudate may occur. Therefore I am very much inclined to accede to Rigal’s opinion, which ascribes to the weakness of the vessels an important role in the coming-about of the oedema. I believe I must further add that in the cases of cyanosis in non-carried-to-term children just adduced by me, the autopsy allowed none of those defects of the foetal blood-circulation to be recognized to which one usually ascribes cyanosis, but only that considerable atelectasis of the lungs was found.

One may object to me that, in the coming-about of the oedemas, the so-called hydraemic constitution of the blood played an important role, and that in the cases of cyanosis without oedema just adduced by me this blood-constitution had to be taken into consideration. But I have by no means left this out of account either: I found in the adduced cases that the blood was in no respect different from the blood as it is usually found in the corpses of those who have died of oedematous sclerema. On the other side, I must say that, although in the majority of cases of oedematous sclerema the blood does not coagulate or yields only a loose coagulum, it yet occurred to me, in rare cases of such a disease, that yellow, fibrinous coagula were to be met with in the left ventricle. Most frequently I observed this where oedematous sclerema had complicated itself with pneumonia; but it also occurred without pneumonia, as without any other inflammation whatever.

It is very important to keep in view the relation in which the weakness of the heart and of the arteries stands to the blood-mass. In general it must be remarked that the children suffering from oedematous sclerema are not seldom full-blooded, which strikes the eye at first glance. All the capillaries, all the veins of them are as it were tense with the fullness of the blood contained in them. Such a pressure on the part of this fluid cannot remain without influence upon the drawing-asunder of the walls of the capillaries and must increase their looseness. It is self-evident that an opposite state must lead to an opposite consequence, namely: with a small mass and hydraemic constitution of the blood, neither its venosity nor its stagnation will lead to oedemas; the child will be blue, but no sclerema will arise. Only from this point of view can the fortunate cures of sclerema through blood-withdrawal be explained, of which, e.g., Valleix and others speak.

The chief task which the therapy of oedematous sclerema must keep in view is: to strengthen the contractions of the heart. If this purpose is attained, then the blood-flow becomes livelier, the stagnations in the capillaries and veins again come into flow, the transudates are resorbed; the blood, through the more rapidly ensuing lesser circulation, experiences to a much greater extent the action of the oxygen, whereby it itself must become a stimulus for the acceleration of the heart-contractions. If the mass of the blood is so great that the heart does not possess the necessary force to set it in motion, then it is comprehensible that blood-withdrawals which press down its mass to the level corresponding to the forces of the heart must exert a favourable action upon the disease as upon the patient.

Let no one, however, suppose that we perhaps recommend blood-withdrawals in oedematous sclerema—this is by no means the case. Only for elucidation of the matter, of its possibly favourable action, have I mentioned blood-withdrawals; but I understand very well that the blood-loss, especially in an infant, can have very evil consequences. We see ourselves the less prompted to blood-withdrawals inasmuch as other, less dangerous means stand at our command, which are capable of heightening the force of the heart-contractions. Among the internal means belong here the so-called excitantia; among the external, especially warmth. All curative means of this kind strengthen the contractions of the heart and accelerate the blood-flow. In the Moscow Foundling Hospital, for the purpose of warming non-carried-to-term children, metal cradles with double walls are employed, in whose interspace warm water is continuously maintained. This kind of application of warmth we recommend especially in oedematous sclerema. In order to acquaint the reader more closely with the effect of the mentioned warming-apparatus, I will briefly communicate the following case:

On the 14th of January, a child born in the 8th month, not carried to term, was admitted into the house, in whom the calves showed a considerable hardness. This hardness spread—though not in the same degree of intensity—over the thighs, the glutei, and the lower part of the belly; the upper extremities and the face were only inconsiderably indurated. The child had a blue-red coloration, was cold, and showed all the remaining marks of oedematous sclerema. On the same day it was placed in the warming-apparatus. On the 15th of January the lower extremities had considerably diminished, the skin of them even wrinkled into small folds, the hardness had everywhere decreased; the child is warm, takes the breast better, gains a more yellowish skin-colour. On the 18th of January the lower extremities had become thin to the degree that they resembled those of an atrophic child; the skin had not only wrinkled into larger folds but even allowed itself to be rolled up; on the remaining parts of the body too the hardness had everywhere disappeared, the skin-colour was yellowish, the temperature normal, the respiration regular, the heart-contractions had become livelier; the child took the breast well and cried with a strong voice. In the following days it gradually began to develop more and more, and the sclerema did not return.

From what has been said, it becomes comprehensible why oedematous sclerema most frequently appears in winter, as in general in the colder season of the year. The cold acts depressingly upon the organs of the circulation and further weakens their—in non-carried-to-term children already weak—function; the warm season of the year, by contrast, acts stimulatingly upon this function and thereby contains conditions which counteract the stagnation of the blood in the veins and capillaries, and hence the formation of oedemas. There is no necessity whatever, for the origination of oedematous sclerema, to invoke a chill (Erkältung), whose action in this case remains still very problematic.

Similarly to the warmth acts also the kneading (massage) of the sclerematous parts, although its action is not a direct one and this method does not possess the same efficacy as the warmth. Inasmuch as we warm the patient and thereby increase the heart-contractions, we accelerate the blood-flow and resolve the blood-stagnation in all parts; through the kneading of the indurated places, on the other hand, we accelerate the blood-flow only locally in these, inasmuch as we move on the blood stagnating in the veins just as the voluntary contractions of the muscles support the venous blood-circulation in the lower extremities; but upon the stagnation of the blood in the internal parts the pressing and kneading can have no influence. To be sure, under these manipulations the child cries from pain, whereby the respiration becomes deeper, the oxidation of the blood in the lungs more complete, and this now can itself exert a stimulating influence upon the circulatory organs. In any case, however, the action of this method is weaker than the direct action of warmth, wherefore one need not wonder, as some physicians have done, that when the sclerema is indeed lifted after the kneading, the patient nevertheless dies. The anatomical changes of the internal organs in oedematous sclerema, which we shall describe presently, show clearly how little the kneading of the feet or hands could exert an influence upon them.¹

¹ Inasmuch as we have presented the physiological foundations for the therapeutic treatment of oedematous sclerema in general outlines, we hold it superfluous to enter more closely into any particular details. Everyone will readily perceive of himself what changes are to be made in the hitherto current method of cure, and in what manner it is to be perfected. All depends on preserving the child so long until—in consequence of its progressing development—the heart gains the necessary force to establish a regular blood-movement in the organism.

The anatomical changes of the internal organs which one perceives in oedematous sclerema can be reduced to three chief forms: 1. to hyperaemias (of passive character), 2. to serous transudates, and 3. to haemorrhages. Accordingly we find:

1. In the cranial cavity. Hyperaemia of the brain occurs seldom in the degree in which one frequently observes it in adults; it is either very inconsiderable, and that for the most part, or is seemingly quite absent. The cause of this lies in the fact that the brain—in oedematous sclerema—usually finds itself in the state of considerable oedema, therefore swells up, and thus mechanically presses the blood out of the capillaries; on the other hand, one often finds the choroid plexuses hyperaemic and still more frequently the pia mater; the veins of the latter especially show themselves considerably overfilled, and frequently one can, by an incision into them, convince oneself of the constitution of the blood in the rest of the corpse—i.e. the blood in them is either fluid, or contains black, loose coagula, or else it is jelly-like (resembling soft fruit-jelly). The sinuses of the dura mater also contain much blood, which bears no less the constitution just mentioned; likewise the fine vessels of the dura mater are in a state of hyperaemia. At times, however, the hyperaemia in the brain itself is not inconsiderable, especially in its cortical substance. In one case we observed such an overfilling of the latter with blood that the whole surface of the cerebral hemispheres appeared throughout of a dark-red colour.

A more constant phenomenon in the organs contained in the cranial cavity is the oedema, so that it is not, say, observed only in the rarer cases. Most frequently the brain itself is oedematous, more seldom the meninges, and still more seldom does one meet with serous exudations in the cerebral cavities. The oedematous brain usually swells up more or less, so that its convolutions (gyri) appear effaced and the dura mater very tense (of which one can convince oneself by an opening made in the parietal bones); such a brain appears at the cut-surface glossy, and the cut-surface itself moist. In the meninges the phenomena of oedema strike the eye still more. In lighter cases the accumulation of a fluid or gelatinous mass fills the convolutions (gyri) of the brain; in other cases the accumulation of serous fluid in the spaces under the arachnoid is so considerable that the arachnoid stands off at single places and rises up in wads which appear filled with fluid. This latter is now bright, translucent, of yellowish colour, more or less watery, now also of gelatinous consistency. In the cerebral cavities the serous accumulation is at times inconsiderable, at times again considerable.

Haemorrhages into the substance of the great brain occur very seldom, and I have observed such a one only once in the cerebellum; more frequently haemorrhages into the tissue of the choroid plexuses and of the pia mater are met with, and still more frequently blood appears admixed to the serous effusions in the cerebral ventricles, or fills the spaces under the arachnoid; in the latter case the fluid met with is either pure blood, or only lightly coloured by admixed blood. Upon a haemorrhage that has taken place one can conclude only from the presence of red blood-corpuscles swimming in it.

In consequence of the oedema, the density of the brain diminishes and it appears softened. This softening reaches at times such a degree that the brain—at the opening of the corpse—wells out of the skull as a half-fluid mass. The softening is always a general one (not restricted to single foci), and the colour of the softened brain always white.

2. In the thoracic cavity. The pleura, or, more precisely to speak, the cellular tissue lying beneath it, appears not frequently in a state of considerable hyperaemia; on the other hand, serous effusions into its cavity are so much the more frequently observed. The quantity of serum is here very various, at times very slight, at other times, however, also very considerable; the serum itself appears now bright and translucent, now reddish from admixed blood and opaque. The haemorrhages appear either as ecchymoses into the cellular tissue lying beneath the pleura, or as free effusion into the thoracic cavity; in the latter case the quantity of blood is often very considerable.

In the lungs the hyperaemia is a very ordinary phenomenon and appears here under the form of hypostasis, i.e. the blood-accumulation occupies pre-eminently the posterior lung-portions, while the anterior find themselves either in a normal or even in a state of anaemia. The parts affected by the hypostasis are dark, hard, and might give occasion to the assumption of a hypostatic pneumonia; but—apart from the fact that the excised, condensed particles of these lung-portions, after careful expression and washing-out, appear normal—the hypostatized parts are distinguished from a pneumonia already by this, that they—laid directly into water—float upon it. Through this last circumstance this hypostatic condensation is distinguished also from atelectasis, inasmuch as the particles taken from an atelectatic lung float in water only when they have first been inflated with air.

Pulmonary oedema is a very frequent phenomenon; it likewise occupies especially the posterior lung-portions and is commonly accompanied by hyperaemia.

Haemorrhages occur not under the form of haemorrhagic infarcts, but arise likewise in the hyperaemic, posterior lung-portions, through rupture of vessels. Through the bronchi the blood escapes into the windpipe, and here it presents itself pure and unmixed. If at the same time oedema is present, then the bloodily coloured serous fluid either wells forth at an incision into the lungs, or passes of itself into the windpipe in order to flow off through mouth and nose of the corpse, or is driven out of the lung-tissue only by pressure with the back of the scalpel.

I will here not speak of the pneumonia and the atelectasis which at times accompany sclerema. I will only remark that the former is very seldom observed, and that West is right when he maintains that what the authors take for pneumonia in sclerema is in the majority of cases nothing other than atelectasis of the lungs.

The heart in oedematous sclerema undergoes less frequently than other organs a change, which expresses itself at most through oedema of its walls. In this case the heart-walls lose their hardness and elasticity and feel doughy; their coloration appears paler than in the normal state, unless perhaps the endocardium is imbibed with blood; the consistency of the oedematous heart-walls is diminished.

The pericardium not seldom contains a greater or lesser quantity of serum, which appears either bright and translucent or—from admixed blood—coloured red. Under the leaf of the pericardium lying against the heart, one not seldom finds ecchymoses. The cellular tissue situated in the anterior mediastinum is not found oedematously distended.

3. In the abdominal cavity. Serous accumulations occur in the abdominal cavity often enough, but their quantity is various, often very considerable, often insignificant; the exuded serum is now pure, now coloured by blood. There is at times also perceived an effusion of pure blood into the abdominal cavity; in such a case the peritoneum, bowels, and stomach appear at times (at the beginning of putrefaction of the corpse) imbibed with blood.

Hyperaemia occurs in the bowels and stomach not frequently, in the latter at most as a cadaveric phenomenon (hypostasis); much more frequently oedema of their walls is observed. Haemorrhages into the internal space of the stomach and bowels I have not once been able to discover, although ecchymoses under their serous covering have at times occurred. In a high degree of the oedema the consistency of the intestinal walls is diminished.

In the liver, both hyperaemia—in a lesser or higher degree—and haemorrhage in smaller or larger foci, and finally oedema, are observed. The latter occurs either in the parenchyma of the liver, or beneath the peritoneum enveloping the liver, which then stands off from the liver, and from which the gland allows itself to be easily shelled out. Similar changes are also suffered by the spleen.

The kidneys show, as regards the anatomical changes, great resemblance to the liver. There occurs in them hyperaemia as well as haemorrhage in the form of ecchymoses under the capsule and in the Malpighian corpuscles, and no less oedema, which has its seat either in the tissue itself or beneath the capsule, the latter being then easily to be separated from the gland itself.

There occurs in oedematous sclerema yet another anatomical change in certain internal organs, which I cannot, however, resolve to set in a necessary and exclusive relation to this disease-form. I am rather inclined to regard it as belonging to those forms in which oedematous sclerema appears in common with the puerperal processes of which I have just made mention. This anatomical change is softening (Erweichung).

The organs most frequently subject to softening are: the liver, the spleen, the kidneys, and the heart. The degree of softening is various—beginning from an inconsiderable loosening of the tissue of these organs up to the complete disintegration into a pulpy mass—which latter I have observed especially in the liver and the kidneys. In one case, where the liver appeared tightly enclosed by its capsule, the tissue of the liver forced its way out with power at an incision through the latter. A like phenomenon I also had occasion to observe in the kidneys; in one case the kidneys, when one took them between the fingers, allowed a distinct fluctuation to be perceived, as though the content of the renal capsule, like a fluid, moved to and fro. After incision of the capsule, the renal substance came forth in the shape of a grey, lightly bloodily coloured, thick, tough, thread-drawing mass. In both just-mentioned cases the corpses still showed no considerable degree of putrefaction, and only a bloody imbibition of certain membranous organs pointed to the beginning decomposition. The softening of the liver and kidneys was always accompanied by oedema, and not once by considerable haemorrhage. In the heart, the softening expresses itself through a diminished consistency of its muscle; it is in the majority of cases not considerable, yet in one case it was so far advanced that a light pressure of the finger pierced through the septum ventriculorum. Least of all was the softening, in the cases of it that occurred to me, marked in the spleen, and this had not once reached that degree of softening observed in the liver and the kidneys.

The microscopic characters of the softening are various according to its degree. In a slight degree the histological elements are preserved and appear only serously infiltrated; they are somewhat swollen and have lost the purity of their contours; in a higher degree the cellular elements are fallen into a fatty degeneration, and although the tissues have yet preserved their shape, they appeared—on account of their turbidity—less distinct. In the highest degree of softening, where the organs are already subject to disintegration, the histological elements are no longer at all to be recognized; everything is transformed into a fine-granular mass in which one perceives fat-droplets and here and there fatty-degenerated cells.

If one now takes into consideration Buhl’s opinion, according to which the elements of decomposition, the oedematous transudates in the puerperal processes of the newborn, passing anew into the blood-vessels, are carried on into all parts of the body and, escaping together with the blood-serum into the healthy organs and tissues, there call forth secondary inflammations—then one must, when one takes into consideration the softenings I have described, always coinciding with oedema, necessarily arrive at the conclusion: that the oedematous transudate in this case becomes an irritant (Irritamentum), which conditions that peculiar parenchymatous inflammation of which Buhl speaks.

The Fat-Sclerema of Infants (Fett-Sclerem der Säuglinge)

The earlier writers assumed, as is known, two kinds of sclerema: the oedematous and the fatty. The authors of the present, especially the German, content themselves with a single one, namely the oedematous form, under which name they describe sclerema in general. I hold it necessary to premise here some notes on the adipose sclerema. Billard, to whom everyone believes he must appeal when he speaks of this form of sclerema, holds this form to be almost only a cadaveric phenomenon. The notes which he communicates in his Traité des maladies des enfants reduce themselves to the following: After he has mentioned that the induration of the cellular tissue presents two different forms—concerning, 1., the cellular tissue itself, and 2., the fatty tissue—Billard says that the induration of the fatty tissue is accompanied by a general infiltration of the subcutaneous cellular tissue: the cheeks, the glutei, the calves, and the back are the usual seat of the induration. It may be joined with an impairment of the blood-flow and of the respiration, or not. Usually it appears in the moments of the agony of the child, yet Billard saw it appear also after death, in corpses of rapidly deceased children. When one cuts through the fatty tissue, it appears hard, dense, like real, coagulated fat; in a word, it has the consistency of the fat of animals slaughtered on the shambles. It is known, Billard concludes, that the fatty tissue is able, under given conditions, to coagulate even during life, when the animal warmth is withdrawn from the living body from any cause.

From this it is to be seen that Billard ascribes the appearance of the fat-sclerema to the coagulation of the subcutaneous fat, conditioned by a sunken temperature of the body. According to his opinion, then, the fat-sclerema is a purely physical phenomenon, explained by the property of the fat to coagulate or melt at certain temperatures.

Somewhat more fully does Valleix enlarge upon the fat-sclerema. This writer too will have observed that the sclerema adiposum develops only in the moments of the agony, and appeals, in order to demonstrate the correctness of this fact, to the observations of Underwood.

The parts most frequently affected, according to Valleix, by the adipose sclerema are: the cheeks, the upper extremities, the back, the glutei, and the anterior portions of the chest; the parts most frequently affected by oedematous sclerema are supposed to remain free of the adipose, so that when both forms occur in one and the same subject, the places occupied by the oedema present no induration of the fatty tissue, and vice versa.

This induration of the fat-rich parts—e.g., of the cheeks, glutei—seemed so surprising that one at first believed special fat-masses must accumulate here. Andry, who found on each cheek, over the musculus buccinator, a lump of hard fat which had the size and form of an almond, regarded it as a pathological product. But anatomy has demonstrated that such fat-lumps are found also in the healthy state. Denis sought the fat-induration in an abnormal reproduction of the fat; on the other hand, Valleix, through careful observation, convinced himself that the quantity of the fat is by no means increased, and that it has only gained in density.

The colour of the skin in fat-sclerema is not violet (bluish), but white or yellow, like wax; the skin cannot be shoved to and fro over the parts lying beneath it and can only with difficulty be raised into a fold, wherefore one was of the view that it was grown together with the underlying parts, indeed even with the bones (Underwood). Among the remaining symptoms are noteworthy: sunken temperature of the body and a peculiar whimpering of the children (similar to that perceived in oedematous sclerema); the one as well as the other of these symptoms was ascribed to the weakness and exhaustion taking place in the last moments of life. Auvity held that the indurated skin could well impair the activity of the muscles of the thorax and thereby the respiration, wherein he found an explanation of the peculiar whimpering.

The anatomical phenomena are, in oedematous and in fat-sclerema, very different. According to Velpeau, the fatty tissue is separated from the aponeurosis by a very distinct layer of a lamellar tissue. When we make a deep incision into an oedematous extremity, we perceive three distinct layers: the actual skin, the panniculus adiposus, and the lamellar layer, which last appears much more considerable than the first two, is at times half an inch thick, and from which a considerable quantity of serum flows out. In fat-sclerema, on the contrary, this last layer appears very thin, white, and dry, and when one tears it, it takes on the shape of fibres. The panniculus adiposus appears in the former case thin, although it is in fact not thinned, of red-yellowish colour, while its fat-masses are separated by oedematous interstices; in the second case it is by no means thinned and looks very white.

In oedema the skin is soft and contains a proper quantity of blood, which at the cutting-through of the vessels wells forth in large, black drops; in fat-sclerema the skin is taut and hard, and the blood emerges at an incision only in scanty, small drops.

In order to give a better characterization of the fat-sclerema, it will not be superfluous to communicate, from Mr. Valleix’s treatise, that passage where he speaks of the oedematous sclerema complicated by intestinal and lung inflammation: “In rare cases Valleix saw that, some days after the oedematous sclerema had developed, an inflammation of the intestinal canal set in. Constipation alternated with diarrhoea, the skin became pale, the oedema disappeared for the greater part, and the children became, in a couple of days, exceedingly emaciated. The skin was no longer reddish-brown, but presented at single places bluish, scorbutus-like spots, and the extremities showed a hardness very different from the former; this was the fat-induration which precedes death. When lung-inflammation was added to the oedematous sclerema, then—under continuance of the oedema—the impairment of the breathing increased, and fat-induration set in, with exclusion, however, of all those parts which had already been the seat of the oedema.”

If the fat-sclerema is not to be an independent disease, but is only to accompany other affections as a symptom, then the question arises: to what disorders does it especially attach itself? Valleix (and likewise Bouchut) are of the opinion that it appears in general toward the end of more or less protracted diseases; in particular, however, Valleix, relying on his own as on others’ observations, names only: 1. thrush (Soor), 2. enteritis, 3. gastro-enteritis, and 4. pneumonia. In the Moscow Foundling Hospital fat-sclerema occurs seldom, wherefore what I have the intention of communicating cannot lay claim to absolute certainty. Nevertheless I hope that the communications I am about to make will not be without interest.

I have observed fat-sclerema as a complication, or, better said, as the outcome of two disease-forms: of diarrhoea and of lung-inflammation, where it appeared not at the beginning, but rather toward the end of the illness. Notwithstanding, my observations do not entitle me to regard it exclusively as a phenomenon of the agony and to reduce it to a coagulation of the fat in consequence of sunken body-warmth. The non-independent appearance of the forms of sclerema adiposum observed by me does not permit the symptoms peculiarly belonging to it to be established, for, appearing together with diarrhoea and lung-inflammation, its symptoms have mingled intimately with those of the named diseases. The fat-sclerema is not the accompaniment of such a diarrhoea as underlies the familiar constant anatomical change of the intestinal canal; it occurs also in catarrhal, follicular, croupous, and diphtheritic affections of the bowels. Therefore the fat-sclerema is preceded—besides those frequent, quantitatively increased evacuations of a familiar peculiar constitution—not seldom also by colics, or these accompany it, in consequence of which the child at first cries much, later—when the strength has already sunk—only groans, whereby the face is wrinkled from pain, and the voice itself sounds weak and hoarse. At first, before the sclerema has yet formed, the child draws—as is usually the case in colic—the little legs up against the body; but after the sclerema has formed, the extremities remain mostly in a half-bent position. If the sclerema extends to the upper extremities, then the movement of these too is impaired. The respiration is at first (in diarrhoea) not yet difficult, but becomes so as soon as sclerema sets in; the child then breathes with effort and with greater frequency than usual. The pulse is seldom accelerated; with normal or retarded rhythm it loses, in the majority of cases—so far as one can judge of it after weakening of the heart-sounds—relatively in its tension. The urine-secretion proceeds more seldom and in lesser quantity. The nutrition is scanty, and the child falls rapidly into a state of marasmus. I had occasion to find a child which I had seen full and round the evening before, the next morning, together with the appearance of the sclerema, with incredibly thin, as it were withered extremities. At the onset of the collapse the frontal fontanelle appeared often sunken. Throughout the whole course of the disease the children suck badly, indeed toward the end of it take the breast no more at all. The mucous membrane not only of the mouth but also of the nose appears dry. In the majority of cases the animal warmth sinks with the onset of the sclerema; yet I have observed a case where the temperature—in a sclerema lasting several days—kept constantly at the height of 30° R.

As soon as the sclerema has formed, the parts affected by it feel hard, whereby the skin—in the majority of cases—looks pale; at times, however, the indurated places take on a bluish shade, e.g. the cheeks, the soles of the feet; in general the skin of the indurated places is dry and rough. According to my observation, the fat-sclerema most frequently affects the lower extremities and the cheeks; thereafter follow the upper extremities and the other parts of the body named by Valleix. In single cases the fat-sclerema was generally distributed, with the exception of those parts which in the normal state allow a deficiency of subcutaneous fat to be perceived.

Where fat-sclerema attaches itself to pneumonia, the latter shows—up to the appearance of the former—the ordinary symptoms peculiar to it. In all cases that presented themselves to our observation, the temperature of the body sank below the norm with the onset of the sclerema, notwithstanding that the pneumonia was in two cases even complicated with pleurisy. The cyanotic coloration of the extremities and especially of the face occurs here more frequently than in those cases where the sclerema accompanies a diarrhoea.

In one case my observation concerned a non-carried-to-term child, come into the world in the 8th month, which presented the signs of an oedematous sclerema of the extremities. At its admission into the infirmary the temperature of the body was 26.9° R. After four days, with the spread of the sclerema over the cheeks, the temperature rose to 29.6° R.; on the same day the child died. At the autopsy no oedema was found in the subcutaneous cellular tissue of the hands, feet, and cheeks, and their induration was rather dependent on the condensed, as it were coagulated fat, which was here present in great quantity. On the other hand, oedema was found in the cellular tissue of the abdominal walls, of the mediastinum, of the lungs, in the brain and the meninges. The bowels showed nothing peculiar, the lungs no condensations. The blood was fluid and black.

In the corpses of children afflicted with fat-sclerema I found almost always the panniculus adiposus very thick, dense, and white. This whiteness it owed doubtless in great part to a considerable anaemia of the tissue; at the same time the indurated parts were distinguished by an unusual dryness, so that from them not the least serous moisture could be expressed. The fat presented a densely coherent mass, and no fat-globules were to be perceived in the layer, such as usually occur in oedematous sclerema. Among the remaining anatomical phenomena—with exclusion of those which belong to the above-named diseases as peculiar signs—I believe I must still bring out that in some cases I met with a considerable hyperaemia of the spleen, in consequence of which this organ had gained considerably in size.

The microscopic investigations of the subcutaneous fatty tissue prove that it has preserved its histological peculiarities, with the difference, however, that the fat-cells are present in it in great superfluity and their circumference appears enlarged. Often there exists such a hyperplasia of the fat-cells at the cost of the fibre-bundles of the cellular tissue, which in such a case appear very thinned; at times the development of the fat-cells is so excessive that the fibre-bundles almost entirely disappear. In general the relation of the fibre-bundles to the fat-cells can be established thus: where the fibre-bundles are broad, there the fat-cells have a lesser circumference, even if they are present in great quantity; where the bundles appear narrow, there the circumference of the cells is greater, and the dense accumulation of them gives the tissue such an appearance that the fibre-bundles appear as if compressed. To outward view, these fat-cells are in nothing distinguished from the fat-cells of the normal fatty tissue, except that they, as said above, show a greater circumference. The remaining layers of the general integuments diverge in no respect from the norm, with the exception perhaps that in the depth of the connective-tissue layer, where it lies upon the aponeuroses of the muscles, a slight division of the cells is perceived.¹

¹ Such a division of the cells I hold—as I have already remarked above—not to be a pathological phenomenon, and still less a pathological phenomenon peculiar to the fat-sclerema. Such a division one perceives, so far as I have seen, everywhere in the corpses of the children and it is only the expression of the physiological growth of the tissues. In any case there is here no resemblance to that type of proliferation of the connective tissue which one meets with in the sclerema of adults, and where the hyperplasia of the fibre-bundles of the tissue occurs at the cost of the atrophying fat-cells, or—better said—at the cost of their disappearance. (See, e.g., the case of Förster in Würzburg. Med. Zeitschr. II. 1861.)

Does the constitution of the fat hereby change? The majority of writers do not admit this and derive the induration of the fat from its coagulation in consequence of a sunken temperature. Mildner, however, believes the contrary, although he does not express himself distinctly wherein the change of the constitution of the fat properly consists. Comprehensibly, this question can be decided only by chemical analysis. In an exquisite case of fat-sclerema I requested the laboratory assistant of the Moscow University, Mr. Zhukowsky, to investigate the skin with the underlying fat-layer. In a piece of fat of 5 grammes he found:

or, expressed in percent:

(The percentage figures as printed in the original do not internally reconcile with the gramme figures; I have reproduced them exactly as they stand in the source, since the arithmetic appears to be an original typographic error.)

Here especially noteworthy is the fat soluble only in boiling ether; such a fat never occurs in the normal state in the subcutaneous fatty tissue. Separated from the other constituents, it presented a white powder which—warmed over the fire—melted. If the mentioned kind of fat occurred in all cases of fat-sclerema, then the induration could most naturally be ascribed to its presence; unfortunately, however, its presence is no constant phenomenon, and the chemical analysis of two pieces of skin taken from corpses of other children who had died of fat-sclerema did not demonstrate the presence of the fat soluble only in boiling ether. Reagents applied by me repeatedly for amyloid transformation have yielded only negative results.

Would there then still remain in force the opinion that the coagulation of the subcutaneous fat, on account of sunken body-warmth, underlies the fat-sclerema? But the question arises whether this is really so. Some writers go so far in this respect as to hold the fat-sclerema downright to be a cadaveric phenomenon. Thus Vogel maintains that the induration of the fat is observed only when the corpses have been exposed to the influence of cold. Even Valleix—who reproaches Denis and Blanche for having sought the fat-sclerema in living children—finds it very natural that they saw it only seldom. “If we”—he continues—”wished to seek it in corpses of such children as had lain, especially in winter, through the night in the anatomical theatre, then we would see that fat-sclerema occurs very frequently.” Finally, Valleix refers to Billard, who found, in the corpses of children still lying in bed, the extremities soft and flexible, but later—after they had already been brought into the anatomical theatre—found these same extremities hard and stiff. Involuntarily one here asks whether Vogel and Valleix have not confused rigor mortis with fat-sclerema, and where then the boundary lies at which the one ceases and the other begins.

But there are also writers who by no means hold the fat-sclerema to be a cadaveric phenomenon and reject the opinion which sees in it only an effect of the agony. Thus, e.g., Rilliet and Barthez—who hold Valleix and Billard’s opinions to be too exclusive—maintain that the fat-sclerema is in some cases the anatomical phenomenon of a proper, well-defined disease. Up to a certain degree the duration of the illness can here serve us as a guide; for if the child suffering from fat-sclerema lives some days, then obviously the induration cannot be designated as a cadaveric phenomenon; on the other hand, such a child can also hardly be assumed to be in the state of agony; for even if the disease is a fatal one, it is yet not therefore an agony, since in that case the oedematous sclerema too would have to be called an agony. As there, so here too, we see a decay of the strength, a weakening of all functions, a sinking of the body-warmth. With such an extension of the concept we would at last come to calling even the tuberculosis dragging on for weeks an agony. That the fat-sclerema is a secondary affection, and is wont to appear in certain diseases as a sign of their fatal outcome, again proves nothing, because tuberculosis too is in certain cases a secondary affection and appears as a sign of a fatal outcome of the pneumonia caseosa.

It is not at all to be denied that the fat-sclerema—in the majority of cases—appears only a few hours before death; but there are also cases where it lasts several days—thus I saw it once endure 5, another time 4 days. Trousseau observed in the Hôpital Necker a child which—already admitted with an indurated skin of the whole body—still lived there two days: it was hard as stone to the touch, and cold, and a pressure upon the indurated places with the finger left not the least impression behind. At the autopsy the panniculus adiposus was found similar to a piece of raw, coagulated fat, and containing not one drop of serous fluid.

The cases adduced above—where the fat-sclerema formed once at a body-temperature of 30° R., and another time where the body-warmth, with existing induration of the cellular tissue, rose from 26° to 29°—prove sufficiently that the fat-sclerema cannot everywhere be regarded as a purely physical phenomenon of coagulating fat.

In order to come somewhat nearer to the solution of this question, I will direct attention to a circumstance which cannot remain without influence upon the greater or lesser density of the subcutaneous fatty tissue, namely to the loss of parenchymatous fluid. The fat appears in the sclerema adiposum unusually dry, and when one cuts through the skin in its entire thickness, indeed down to the muscles, one is often not in a position to express a single drop of serous fluid. It is comprehensible how, in consequence of this, the parts lose their turgor and contract, on which their shrinkage and the surprising sudden emaciation of the children depend, which cause them to be held as atrophic, while the autopsy demonstrates beneath their skin whole masses of fat—which was thus present in superabundance. In those cases where the fat-sclerema appears after copious diarrhoeas, or immediately after inner, more considerable oedemas (as took place in the case adduced by me), such a drying-out of the subcutaneous fat-layer is easily comprehensible; for, just as in cholera, the parenchymatous fluid is here absorbed by the vessels in replacement of the blood-serum consumed in the outer or inner exudations.

We said above that some writers have wished to see in the fat-sclerema a heightened fat-reproduction (Denis), while others (Valleix) reject this opinion. The question arises which of these views is the more correct. I must confess that the thickness of the fat-layers under the skin is not always the same; in the majority of cases it is so great that one might in fact think of a heightened fat-production; at times, however, it does not exceed the measure met with in well-nourished children. In polysarcia of adults, fat is, as is known, deposited into the mediastinum, the omentum, the mesentery, around the heart and the kidneys; in the newborn—where one, judging by the thickness of the fat-layers under the skin, would be tempted to think of polysarcia—I have yet never met with such deposits; not even in the mesentery have I observed such a deposit as Steiner and Neureutter (the fatty and amyloid degeneration of the liver in childhood, “Jahrb. f. Kinderheilkunde. 1865. Hft. III.”) found. The only, not considerable fat-deposits I have at times met with in the form of small islets under the pleura. As regards the fatty infiltration of internal organs, one can, as is known, attach to this phenomenon no special importance, because fat occurs very frequently in the liver-cells of the newborn, especially in those who have suffered from catarrh of the intestinal canal and from lung-inflammation, which diseases in turn are wont to precede the fat-sclerema.

Still less is one entitled to set the fat-sclerema in relation to that acute fat-degeneration of the newborn which Buhl has described. With this disease-form the fat-sclerema has not the least resemblance, neither in respect of the symptoms observed during life, nor in respect of the anatomical phenomena found in the corpses.

As is known, one designates by the expression “sclerema” an induration of the skin and of the cellular tissue lying beneath it; yet one finds among the writers indications that under this expression an induration of the muscles too was comprised. And in fact, when one grasps a sclerematous extremity, one feels first a general hardness, whether this depends on a heightened density of the skin and of the subcutaneous cellular tissue for itself, or also of the more deeply lying parts. A closer palpation and shoving of the skin will of course immediately reveal whether it is condensed or not. But when one views the matter from the anatomical standpoint and takes as the starting-point of the sclerema the definite change of the subcutaneous cellular tissue, then there is no ground for the assertion that a similar change cannot also take place in the intermuscular cellular tissue. When, e.g., in fat-sclerema the predominance of the fat and the relation of the actual fatty tissue to the remaining elements of the subcutaneous cellular tissue constitutes the essence of the disease, is then not something similar also observed in the familiar form of muscle-hypertrophy, which depends on a fatty change of the intermuscular cellular tissue and which one at present calls lipomatosis luxurians musculorum progressiva?

The comparative juxtapositions we have just indicated could be made only by the inductive way, and in reality no one has yet seen, in fat-sclerema, a fatty degeneration of the intermuscular cellular tissue; nevertheless the fact cannot be contested that a condensation of the muscles too has been drawn into the domain of sclerema. Thus, e.g., Bouchut says that with the sclerema, in some patients, a stiffness of the whole trunk and head was observed, and appeals in this to Dugès, who claims to have observed such cases where one could lift the children in the air by the head, as though they were formed of one piece of wood. Bouchut, who claims to have perceived, in a child on the eve of its death, the same phenomena, guards himself withal by saying that in this child no brain-symptoms whatever were present. This pointing to brain-symptoms shows clearly that Bouchut had reckoned such a sclerema to the class of tonic cramps, by which without doubt the muscles were affected. The correctness of such a supposition finds its confirmation in his earlier remark, that although the children affected by sclerema preserve the faculty of movement and use their hands and feet fairly freely, at times cramp-like movements yet appear in them and their hands slightly tremble.

I have observed such an, as it were, wood-like stiffness of the muscles only in tetanus of the newborn, in which not the least sclerema of the skin and of the subcutaneous cellular tissue took place; nevertheless the hardness of the extremities, at their touch, awakened the thought of sclerema. Although I have never observed tonic cramps in the course of sclerema, I cannot yet maintain that such never takes place. It may also be that the description of Dugès and Bouchut is correct, only with the reservation that in the cases adduced by them the tetanic contraction of the muscles, and the sclerema of the skin and subcutaneous cellular tissue, each existed for itself.¹

¹ Mildner assures us that after convulsions a heightened density of the general integuments (the skin) remains behind, which at times takes on the form of scleroderma. (Journal für Kinderkrankheiten, Bd. V., p. 391.)

Going farther in this direction, we strike upon Baumès, who sets the essence of the sclerema in a rigidity of the muscles. This rigiditas musculorum can either be a phenomenon of the living body, or be observed in the corpse, where it depends on the coagulation of the myosin (rigor mortis).

Passing over the familiar anatomico-pathological changes which call forth a condensation of the muscles and have nothing in common with sclerema, we will dwell only on the functional muscle-rigidity, and ask ourselves whence it can arise. It is known that in the muscles the so-called tonus (muscle-tonus) prevails, which for its existence requires a regular innervation as a regular nutrition. With heightened innervation the tonus too is heightened; an unusual heightening of the physiological tonus borders on the rigiditas musculorum (Zaradelli). The same takes place with a heightened nutrition of the muscles; but since exercise possesses the greatest influence upon such a heightened nutrition, the muscles most engaged in exercise also appear the hardest. There, where in consequence of pathological conditions the contractility of the muscles that withdraws itself from the influence of the will is heightened, cramps arise, which can be either clonic or tonic. In the former the contraction of the muscles is of short duration, and therefore their condensation too only transient; here, then, there can be no question at all of so persistent a disease as sclerema represents. Otherwise the matter is with tonic cramps, where the contraction of the muscles is more or less sustained and—in consequence thereof—the condensation of the muscles can indeed look somewhat similar to sclerema. Yet the muscles, especially the voluntary ones, belong, as is known, to those tissues which cannot remain in a continuous tension, and upon whose tensing a relaxation usually follows. If one therefore observes, for a longer time, tonically contracted muscles, then it is not difficult to perceive the moment when the tension ceases and the relaxation of the muscles sets in. Self-evidently, such alternating indurations and softenings are not perceived in the indurated skin and in the subcutaneous cellular tissue—and precisely thereby is the induration of the cellular tissue distinguished from the tonic cramps, out of which some authors have wished to make a cramp-form (krampfhafte Form) of the sclerema.

Last Updated on 08/15/26