Degosʼ Disease (Papulosis Atrophicans Maligna): Report of a Case with Degenerative Disease of the Central Nervous System

Malignant atrophic papulosis

“…Nomland et al (i960) noted areas of encephalomalacia at the post mortem of a patient with MAP who died of peritonitis with perforation of the stomach. However, it was Gever et al (1962), Culicchia et al (1962) and Winkelmann et al (1963) who drew attention to the possibility of the central nervous system being affected. In 13 cases found in the literature (17",,) central or peripheral neurological signs were observed, and in 7 cases these dominated the clinical picture.…”

“…Even if the first and the majority ofthe cases were described in Europe, the disease does occur in other continents, particularly in the U.S.A., and it is not only Caucasians who are affected. One single familial incidence was reported by Hall-Smith (1969) in a young girl of 16 who died in 1964 of visceral haemorrhage, and whose mother, at the age of 45, presented with cutaneous lesions.…”