Degos' disease, malignant atrophic papulosis, is a rare disease characterized by a pathognomonic appearance of porcelain-white, atrophic papules with peripheral erythema and multiple occlusive thrombotic vascular pathology. It sometimes involves internal viscera such as the gastrointestinal tract, the central nervous system and kidney which may bring to death. Although several reports showed the associations between Degos' disease and antiphospholipid antibody or systemic lupus erythematosus, its pathogenetic significance remains elusive. The previous 4 Korean cases of Degos's disease involved only skin and/or the gastrointestinal tract. We report a case of systemic lupus erythematosus with Degos' disease involving skin, kidney and small intestine.

Keywords: Malignant atrophic papulosis, Degos' disease, Systemic lupus erythematosus, Antiphospholipid antibody