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October 29, 1960

Lethal Cutaneous and Gastrointestinal Arteriolar Thrombosis(Malignant Atrophying Papulosis of Degos)

JAMA. 1960;174(9):1170-1173. doi:10.1001/jama.1960.03030090026006

The authors report in detail two cases of the rare disease entity "malignant atrophying papulosis." No previous descriptions of this lethal cutaneointestinal syndrome have been found in the American literature. The patients were two men 24 and 30 years of age. The cutaneous lesions consist of pink papules which progress to a characteristic necrotic lesion with porcelain-white center and are diagnostic. The eruption is sooner or later associated with ill-defined gastrointestinal complaints which almost always eventuate into the clinical picture of peritonitis. The outcome is fatal, with death due to multiple intestinal perforations. The etiology is unknown and no satisfactory treatment is available.