Necropsy Findings in a Case of Congenital Scleroderma and Sclerodactylia

Abstract

Today there are two diseases which the dermatologist thinks of as producing a hardening of the skin in very young infants or in the newborn ; these, edema neonatorum and sclerema neonatorum, are so uniformly and routinely described in all the textbooks that they at once come to the mind of every dermatologist when any abnormal cutaneous firmness appears at birth or in early infancy. This is true in spite of their rarity, particularly in the case of sclerema neonatorum, which the case herewith presented most closely resembles, and which is so uncommon that the occurrence even of clinical cases is still held to afford sufficient ground for report in the literature. In reviewing the literature of the last five years, I have found only fourteen such case reports. The unusual title given it and the usefulness of this case depend almost entirely on the fact that a necropsy was performed, and that the subsequent microscopic examination revealed certain features which did not correspond to those published of either of these two dis¬ eases. This led to a search of the relevant literature and further study, with the result that I found another writer, Lieberthal.1 who believes that there is such a condition as scleroderma to be found in the new¬ born, and another, Cockayne,2 who found it in an infant 7 months old. It is to support this idea and to indicate still further that scleroderma may be found (rarely) among infants as well as adults, and also to place on record what I believe to be valuable necropsy findings that this communication is offered. RELATIONSHIP OK CUTANEOUS INDURATICXS It might conduce to clearness if at the outset certain points relating to the above three indurating cutaneous diseases be noted briefly : The first, edema neonatorum, is a disease which our case in no way resembled; at one time it was confounded with the real sclerema