Abstract

Malignant atrophic papulosis (MAP), or Degos Disease, is a rare vasculopathic disorder that involves the skin, gastrointestinal tract, and central nervous system. Diagnosis is made from distinct cutaneous lesions with characteristic findings on histology. However, a variety of pathologic findings have been described in association with this entity. We present a case of a 58-year-old-male who was diagnosed with MAP and the histologic features that led to the diagnosis. Our patient initially presented to the Emergency Department for a penile eschar, and a shave biopsy demonstrated mild stasis changes with hyalinization of dermal vessels. One month later, the patient returned to the hospital with acute abdominal pain. Patient was admitted and placed on intravenous antibiotics and steroids, due to concern for underlying infection and systemic vasculitis. Dermatology was consulted, and on examination, there were scattered diffuse erythematous papules with central white-porcelain scars. Two punch biopsies were performed on representative lesions. One biopsy exhibited dermal mucinosis and vascular occlusion due to intimal proliferation with associated foamy histiocytes. The other biopsy demonstrated a wedge-shaped area of fibrinoid necrosis, extending from the deep dermis to the necrotic epidermis. Our patient was subsequently diagnosed with MAP. While hospitalized, our patient developed bowel perforations, requiring surgical intervention. Eculizumab was started with improvement of disease. This case exemplifies the clinical course of patients with MAP and the histopathologic findings seen on cutaneous specimens. It is important for dermatologists and dermatopathologists to be aware of the various histopathologic characteristics to diagnose these patients early and to initiate treatment.