Degos syndrome: malignant atrophic papulosis

Abstract

Malignant atrophic papulosis is a rare disorder characterized by pathognomonic cutaneous lesions that have been associated with multiple infarctive thrombotic lesions of other viscera, most notably the gastrointestinal tract and the central nervous system. Systemic involvement may develop from weeks to years after the onset of the characteristic cutaneous lesions or, rarely, may precede the cutaneous lesions. However, the existence of patients with a prolonged, purely cutaneous variant of this disease has been increasingly appreciated, and this brings into question the appropriateness of applying the term "malignant" to all patients who have the peculiar characteristic cutaneous lesions of malignant atrophic papulosis. Despite half a century of sporadic investigation, the precise cause of this disease remains unknown, and accurate classification of this entity as a primary vasculopathy or primary coagulopathy has not been possible. Unfortunately, no effective therapy exists for those patients in whom systemic involvement develops.