Piebaldism: rare but all dermatologists have heard this strange term during residency
- Atrophic Malignant Papulosis was described in 1942 by the Parisian dermatologist Dr. Robert Degos (1904-1987) en 1942 in a 49 year old plumber. Biopsies were examined by Dr Achille Civatte and showed marked epidermal atrophy with absence of infiltrate in the dermis, with vascular changes leading to thrombosis. Although extremely rare, Degos was thereafter able to point more than 70 cases in the literature by 1979
- The clinical presentation of this rare condition is so characteristical that it can be recognized even by those who have never seen it before: a small macule of a few millimeters with a central white atrophy which looks as if if were polished porcelain.
- It is a systemic vascular condition which often has a fatal outcome. Systemically the bowels and the brains are infarcted and the intestinal mucosa has similar lesions.
- The Austrian pathologist Köhlmeier described this disease one year before Degos, but it is the latter who defined the syndrome
- Degos was a superlative clinician who also described (independently) in 1954 reticular pigmented anomaly of the flexures: Dowling-Degos disease.
Contributors
Dr Christophe Hsu – dermatologist. Geneva, Switzerland
Source of information:
-Harms M. Dermatologica Helvetica (The Swiss Journal of Dermatology and Venereology: www.dermatologicahelvetica.com
-Crissey JT el al. Dermatology and Dermatologists (2002). Parthenon Publishing
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