Papulosis atrophicans maligna (Köhlmeier-Degos disease): a disseminated occlusive vasculopathy.

[malignant atrophic papulosis]

Malignant atrophic papulosis usually presents as pathognomonic skin lesions followed by acute abdominal pain , bowel perforation , peritonitis , and death . Rare patients who may lack gastrointestinal symptoms present with central nervous system manifestations , including headache , paresthesias , weakness , and rapid deterioration to death . The patient reported here was a 47 -year -old man whose neurological symptoms apparently preceded his cutaneous lesions . His course consisted of a disseminated neurological disease and exacerbated following a herpes zoster infection . His condition rapidly deteriorated despite corticotropin , glucocorticoids , and low -molecular-weight dextran . Necropsy revealed a disseminated occlusive vasculopathy and diffuse encephalomyelomalacia of the brain and spinal cord . A review of autopsied patients with central nervous system involvement is provided .