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

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.