Degos' disease: A rare condition simulating rheumatic diseases

Abstract

Dego's disease is an uncommon thrombo-occlusive vasculopathy that presented with skin rash and thrombotic complications affecting internal organs that may simulate rheumatic diseases and may be brought to the attention of rheumatologists. We present here a case of a middle-aged woman who presented with acute bowel infarction, persistent fever, elevated inflammatory markers and reversed albumin/globulin ratio suspicious of systemic vasculitis clinically. The diagnosis of Dego's disease was made from the classical skin lesions which were pink to brown papules with central depression and surrounding violaceous rim that were distributed over the trunk and extremities. Histology showed typical wedge-shaped infarction in the affected organs with endothelial proliferation and occlusion by thrombus. Our patient was put on aspirin but suffered from recurrent bowel infarction 1.5 years later and eventually succumbed to septic complications. © Clinical Rheumatology 2009.