Degos disease associated with protein S deficiency

Conflicts of interest: none declared. Sir, Malignant atrophic papulosis or Degos disease is a rare, sometimes fatal disorder characterized by multiple infarcts in the skin and internal organs, especially the gastrointestinal tract.1 More recently, typical cutaneous lesions of Degos disease have been documented in patients with a benign clinical course without evidence of systemic disease, and the disorder has been reclassified into benign and malignant forms.2 The pathogenesis of malignant atrophic papulosis remains unknown, but various abnormalities in blood coagulation and autoimmunity have been described.3 We report a case of Degos disease, so far benign, associated with protein S deficiency. A 22‐year‐old woman with clinical history of coeliac disease and allergic asthma was referred for isolated, painful lesions distributed on the trunk and the proximal parts of the extremities. These began on the buttocks, and up to 10 papules then progressively appeared over the following 6 months. Lesions started as fleshy erythematous 0·5–1 cm papules, progressed developing a central necrotic crust, and resolved leaving atrophic scars of porcelain appearance, with a rim of erythema (Fig. 1a). Topical corticosteroids and antibiotics were not effective. She remained well, with no abdominal symptoms or signs of malabsorption, and general examination was otherwise unremarkable.