Malignant Atrophic Papulosis (Degos Disease)

Degos disease, also called malignant atrophic papulosis, is a very rare autosomal dominant microvascular occlusion syndrome. It is a disease involving the internal organs, mainly the gastrointestinal and central nervous systems, and can be fatal. Disease onset is usually between the ages of 20-40 years. In this paper, a 33-year-old female patient with Degos disease was presented. The patient was admitted with the complaint of scabbed wounds particularly on her legs that had been present for the past 10 years. On her dermatologic examination, she had erythematous scabbed papules measuring 3-5 mm on her both legs. In addition, she had multiple white atrophic lesions on both her legs and trunk. Biopsies were taken from old and new lesions. Biopsy results were consistent with Degos disease. Systemic involvement was not detected on her tests. We wanted to present this case in order to demonstrate the histopathological differences between old and new active lesions and review the disease.