Malignant form of atrophic papulosis with lethal abdominal involvement

Atrophic papulosis (Kohlmeier–Degos disease or Degos disease) is a rare occlusive arteriopathy involving small-calibre vessels of the dermis, gastrointestinal tract, central nervous system (CNS) and occasionally other organs. It would appear to be a vasculopathy or an endovasculitis, with a purely cutaneous benign variant and a systemic variant with cutaneous manifestations (malignant atrophic papulosis). Gastrointestinal involvement is the most frequent and lethal systemic complication. This is the most common cause of death, followed, less frequently, by CNS bleeding, and pleural or pericardial involvement.