Lethal Hereditary Bullous Epidermolysis. Survey – and a Typical Case.

Abstract

SUMMARY Lethal hereditary bullous epidermolysis is set up as one of the affections in the group of epidermolyses parallel with simple and dystrophic epidermolysis. It is characterized by bulla formation and epidermolysis with subsequent healing without cicatrization, with frequent bone dystrophy and ungual changes, rapid lethal issue, and familial occurrence. A typical case is described and illustrated. Already at birth the patient presents large epidermolyses. There is found bulla formation beginning with edema and cell infiltration in the uppermost layer of corium. The epidermis is exfoliated and replaced by a new thin atrophic epidermis, with corresponding imperfect development of sebaceous glands and elastic fibers. Hairs and sebaceous glands are atrophic everywhere and, after exfoliation of the epidermis, in further decomposition.In contradistinction to some previously reported findings, milliary cysts form from sebaceous glands. Cultivation from bullae yields no growth. The affection is resistant to any treatment applied. New bullae continue to appear, and after seven months the child dies without signs of infection. Pig. 1 shows the macroscopical look of the affection, whereas Pig. 3 illustrates the typical microscopical changes in the skin.