Abstract | UNLABELLED: 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: 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:
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Authors | Athanasios Theodoridis, Evgenia Makrantonaki, Christos C Zouboulis |
Journal | Orphanet journal of rare diseases
(Orphanet J Rare Dis)
Vol. 8
Pg. 10
(Jan 14 2013)
ISSN: 1750-1172 [Electronic] England |
PMID | 23316694
(Publication Type: Journal Article, Review)
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Chemical References |
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Topics |
- Humans
- Immunosuppressive Agents
(therapeutic use)
- Malignant Atrophic Papulosis
(diagnosis, drug therapy, pathology)
- Prognosis
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