Degos' Disease (Malignant Papulosis)

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Abstract
Three patients with Degos' disease (malignant papulosis) were seen at the Cleveland Clinic. The course of the first patient's disease was typical with cutaneous, gastrointestinal, and systemic involvement and death. The other two patients survived despite the typical cutaneous lesions. Abnormalities of the immunoglobulin fraction IgA were present, as well as an abnormal fibrinogen value. However, there appears to be no direct relationship to periarteritis nodosa or other forms of vasculitis and the cause of Degos' disease remains unknown.

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