Malignant Papulosis with Atrophy (Degos)

Abstract

Enough cases of the fatal cutaneointestinal syndrome malignant papulosis with atrophy have now been reported so that it can be considered a clinical and pathologic entity. When, in 1942, Degos et al. 1 presented their first patient, then having only skin lesions, they were unaware of the future fatal outcome, but nevertheless felt that the patient had a previously undescribed disease of the skin. Several months later the man died after a severe acute gastrointestinal condition. At operation, and later at autopsy, there was no perforation of the intestine, but many yellow lesions of the small intestine were found. These atrophic lesions of the intestine were identifiable, both microscopically and macroscopically with the atrophic skin lesions, and were assumed to have the same causation. The history of development, the character and type of the eruptive lesions are very uniform in all the cases reported. Degos' first case illustrates the course