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Degos-Kohlmeier disease [6, 17,19] is a rare condition (approximately 200 cases published worldwide up to 2009). It was first described in 1941 under the name malignant atrophic papulosis, as a frequently lethal syndrome involving the skin and gastrointestinal tract. It is in fact a systemic vasculitis preferentially affecting the vessels supplying the skin, gastrointestinal tract, central nervous system and eyes. It affects young adults of both sexes (1.3 men/1 woman), most commonly of Caucasian origin. It is rare in infants and children ; its etiopathogenesis remains mysterious and its prognosis difficult to predict. No official guidelines for its treatment exist.
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