Köhlmeier-Degos Disease (malignant atrophic papulosis) and neurologic involvement.

Dr. Felipe Slaviero – Serviço de Neurologia e Neurocirurgia Rua Teixeira Soares 640 99010-901 Passo Fundo RS Brasil. E-mail: [email protected] The malignant atrophic papulosis (MAP) was first described by Köhlmeier in 1941 and recognized as a specific entity by Degos in 1942. This rare disorder is an obstructive vasculopathy of unknown origin, characterized by vascular lesions of the skin, central nervous system (CNS) and gastrointestinal tract. Skin lesions are usually the first manifestation of the disease, although the poor prognosis is resultant of the gastrointestinal and CNS involvement. Once multisystemic disease has developed, death occurs in approximately 50% of patients within 1 to 2 years, mostly due to intestinal perforation or, less commonly, cerebral infarctions. The neurologic manifestations of MAP include cerebral infarcts, subdural collections, venous sinus thrombosis, polyrradiculoneuropathy, and nonspecific symptoms without objective findings. Myelopathy and myopathy are less frequently reported. Since the disease is rare, case reports may contribute to understand its course and response to different approaches. We report a case of delayed diagnosed MAP, highlighting the neurologic involvement, the clinical and histologic patterns to provide its proper diagnosis.