[Malignant atrophic papulosis (Köhlmeier-Degos disease)]. 1989

G Burg, and D Vieluf, and W Stolz, and M Landthaler, and O Braun-Falco
Dermatologische Klinik und Poliklinik, Julius-Maximilians-Universität Würzburg.

On the basis of 106 patients with malignant atrophic papulosis in the literature, including one case of our own, the clinical, histopathological and nosological features of this disease are reviewed. Histopathologically, the typical papules with central porcelain-white atrophy show broad wedge-shaped necrosis of the connective tissue due to thrombotic occlusions of small vessels in the corium. The disease leads to death in 50% of these cases, mostly due to involvement of the gut and the central nervous system. Malignant atrophic papulosis is the prototype of a disease, in which skin lesions are the primary sign of a systemic disorder demanding the interdisciplinary cooperation of various specialists.

UI MeSH Term Description Entries
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D009336 Necrosis The death of cells in an organ or tissue due to disease, injury or failure of the blood supply.
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D001284 Atrophy Decrease in the size of a cell, tissue, organ, or multiple organs, associated with a variety of pathological conditions such as abnormal cellular changes, ischemia, malnutrition, or hormonal changes. Atrophies
D012867 Skin The outer covering of the body that protects it from the environment. It is composed of the DERMIS and the EPIDERMIS.
D012871 Skin Diseases Diseases involving the DERMIS or EPIDERMIS. Dermatoses,Skin and Subcutaneous Tissue Disorders,Dermatosis,Skin Disease
D013919 Thromboangiitis Obliterans A non-atherosclerotic, inflammatory thrombotic disease that commonly involves small and medium-sized arteries or veins in the extremities. It is characterized by occlusive THROMBOSIS and FIBROSIS in the vascular wall leading to digital and limb ISCHEMIA and ulcerations. Thromboangiitis obliterans is highly associated with tobacco smoking. Buerger Disease,Buerger's Disease,Thromboangitis Obliterans,Buergers Disease,Disease, Buerger,Disease, Buerger's

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