(Sclerema of the new-born; Scleroderma neonatorum)
Definition. – An induration of the skin and subcutaneous tissues with local and general circulatory disturbances, occurring either at birth or soon after.
Symptoms. – First described by Underwood in 1784, this rare disease may be present at birth or develop within ten days, rarely later. The strictly congenital cases, if not still-born, die within a few days. As a rule the legs are first involved; following this, the back, chest and remaining surfaces of the skin become affected in four or five days. It rarely begins on the face and spreads downward, but whatever its point of origin, the process may be arrested when only part of the surface is involved. The skin affected presents a hard, resisting surface which cannot be pitted on pressure or gathered into folds. At first whitish or dirty yellow in color, it soon becomes deeper colored or livid in hue. The natural wrinkles are obliterated and the surface looks or feels smooth, polished, firm, cold and marbleized. Immobility of the face may be so pronounced as to prevent movement of the jaws. Thus the patient is unable to take food. Often an infant will show a fixation of the whole body so marked that raising it with one hand will not change the straightened outlines. The pulse and respiration may be less than half their normal frequency and the temperature markedly sub-normal, sinking lower as death approaches.
Etiology and Pathology. – Both are obscure. The condition occurs in premature children or those who suffer form malnutrition, faulty circulation, or digestive, pulmonary or cardiac disorders. Whatever the predisposing cause may be, the immediate one is the retardation of the cutaneous capillary circulation. Pathological findings present no unit; Langer believes the disease is due to an infiltration and solidification of subcutaneous fat; Parrot holds that the solidification results from diarrheal depletion; and Ballantyne claims that the disease is due to an over-growth of connective tissue leading to atrophy of the fat-cells, and that the condition is a trophoneurosis.
Prognosis and Treatment. – These cases usually end fatally. Only those in which the induration is not general or complete recovery. Measures that increase the body temperature and improve the circulation of the blood, are indicated. An incubator can be used, if convenient, for the purpose of furnishing artificial heat or hot flannels, cotton, wool and hot-water bottle may be fed by the mouth or if unable to swallow, a rubber tube and syringe or stomach pump can be used to introduce nourishment into the stomach. Indicated drugs can be given on the tongue or by hypodermic injection; likewise stimulants if needed. Apis, Alumina, Bryonia and Secale may be studied.