A 18-year-old male patient's case was diagnosed as Degos'disease with pathognomonic skin lesions, accompanied by small bowel perforation, and reported here. Skin histopothological test show that the typical wedge-shaped necrobiosis and lymphocyte inflammatory infiltration. Vessels showed narrowing and thrombosis, with lymphocyte infiltration. Degos' disease is a systemic necrotizing vasculitis. Skin biopsy can confirm its diagnosis. Severe systemic complication should be prevented. Degos' disease should be considered in the differential diagnosis of skin lesions associated with systemic involvement.
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