Papulosis atrophicans maligna (Köhlmeier‐Degos disease): A disseminated occlusive vasculopathy

• DOI: 10.1002/ana.410030504
• Publication Date: 2005-01-01T13:06:44Z
• Authors: H. Richard McFarland; William G. Wood; Bruce V. Drowns; Antonio C. O. Men...
• Keywords: Pathognomonic; Perforation

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, rapid deterioration to The patient reported here was a 47-year-old man whose neurological apparently preceded his cutaneous lesions. His course consisted of disseminated disease exacerbated following herpes zoster infection. condition rapidly deteriorated despite corticotropin, glucocorticoids, low-molecular-weight dextran. Necropsy revealed occlusive vasculopathy diffuse encephalomyelomalacia the brain spinal cord. A review autopsied involvement is provided.