Malignant atrophic papulosis (Köhlmeier-Degos disease) - A review

DEFINITION OF THE DISEASE: Malignant atrophic papulosis (MAP), described independently by Köhlmeier and Degos et al., is a rare, chronic, thrombo-obliterative vasculopathy characterized by papular skin lesions with central porcelain-white atrophy and surrounding teleangiectatic rim. EPIDEMIOLOGY: Less than 200 cases have been described in the literature. The first manifestation of MAP usually occurs between the 20th and 50th year of life. CLINICAL DESCRIPTION: The cutaneous clinical picture is almost pathognomonic. The histology is not consistent but in most cases it shows a wedge-shaped connective tissue necrosis in the deep corium due to a thrombotic occlusion of the small arteries. In the systemic variant, manifestations mostly occur at the intestine and central nervous system. ETIOLOGY: The etiopathogenesis of the disease remains unknown, a genetic predisposition may occur. Vasculitis, coagulopathy or primary dysfunction of the endothelial cells have been implicated. DIAGNOSTIC METHODS: Diagnosis is only based on the characteristic skin lesions. DIFFERRENTIAL DIAGNOSIS: It depends on the clinical presentation of MAP, but systemic lupus erythematosus and other connective tissue diseases need to be considered. MANAGEMENT: No effective treatment exists for the systemic manifestations, while compounds that facilitate blood perfusion have achieved a partial regression of the skin lesions in single cases. PROGNOSIS: An apparently idiopathic, monosymptomatic, cutaneous, benign variant and a progressive, visceral one with approx. 50% lethality within 2–3 years have been reported. Systemic manifestations can develop years after the occurrence of skin lesions leading to bowel perforation and peritonitis, thrombosis of the cerebral arteries or massive intracerebral hemorrhage, meningitis, encephalitis, radiculopathy, myelitis.