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Sclerema

children, skin, knopfelmacher, newborn, lower and extremities

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SCLEREMA Clinical onset of sclerenia is similar to that of sclercedema in that it affects the lower extremities, especially the calves, in a symmetrical arrangement. On careful palpation even in the early stages of the disease, a doughy sensation can be felt in the deeper layers of the skin. This soon extends over the thighs, trunk, and neck. The head and upper extremities are the last to be involved. The penis, scrotum, soles of the feet and palms of the hands remain free. The induration increases so that the skin gives a sensation of board-like resistance and coldness. Various descriptions of the color of the skin are given by different authors. Neumann describes it as yellowish white and waxen; Heubner as grayish brown; and Parrot as light blue and cyanotic. Small ecchymoses may occasionally be found on the lower extremities.

The skin is immovable and hard and cannot he raised from the underlying tissue. In contradistinction to sclercedema the affected parts become atrophied. The legs are rigid and the children are motionless like a stick of wood. The face has a mask-like appearance.

The general condition is bad, the weight falls rapidly and the tempera ture is constantly subnormal and may reach 30° C. (S6° F.) or lower. Nurs ing and feeding are difficult on account of the rigidity. The mucous mem branes are extremely dry. The respi ration is lowered to 16 or even less a minute and the pulse falls to 50 or 30. Older children will give a shrill cry (cri de detresse) at frequent in tervals. Convulsions may occur. The sleep is generally disturbed. The amount of urine is di mi ni shed and has a heavy sediment. Albumin is absent. The bowels often do not move spon taneously. The child generally dies in a coma after a few clays. The course is the more rapid and fatal the younger the infant. The author has bad cases of infantile atrophy in which this dis ease ran a prolonged course lasting weeks and ending in recovery.

in contrast to selercedema, is a very rare affection. It occurs not only during the first clays of life but also up to the first. three months (Berthod) in debilitated, premature and poorly

nourished infants. Knopfelmacher puts the age-limit for its occurrence at the sixth month. It is observed more often in summer, since it occurs, especially in older nurslings, as a sequelum of cholera infantum and chronic catarrhal enteritis in the stage of atrophy (Parrot, Henoch). Congenital cases do occur, however, although with ex treme rarity.

Nature and Pathogenesis.—According to Luithlen one must dif ferentiate two forms of sclerema; one apparently autochthonous, that is occurring independent of any other disease; likewise a second form developing as the result of profuse losses of plasma or as a consequence of effusions into body-cavities (pleuritis, internal bleedings). This author assigns more of a symptomatic role to sclerema.

Essentially the process consists in a drying up of the body (Cle mentowsky, Widerhofer, Soltmann). The peculiar composition of the fat in the newborn and young nursling according to the investigations of Langer and Knopfelmacher seems to furnish the basis for the oc currence of sclerema. The fat of a newborn contains only 43.3 per cent. of oleic acid (Knopfelmacher) against 6.3.0 per cent. in the adult and in older children (Langer); whereas the amount of palinitic and stearic acid is greater (31:10) in the young nursling and congelation takes place at a higher temperature than in older children.

Later researches (Thiemich and Siegert) have left these findings again in doubt.

The lowering of the external temperature must play an etiolog ical part also in sclerema, however not directly through the effect of the cold but indirectly by unfavorably affecting the respiration and circulation. Cases occurring without previous fluid losses and without the influence of cold are rare and etiologically totally obscure. A few authors (Schmidt., Aufrecht) have assumed an infectious origin for sclerema although the adduced bacterial findings only go to show that sclerema can occur after or with septic disease in the newborn.

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