Published in:
Open Access 01-12-2013 | Review
Malignant atrophic papulosis (Köhlmeier-Degos disease) - A review
Published in: Orphanet Journal of Rare Diseases | Issue 1/2013
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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.