A case of systemic malignant atrophic papulosis (Köhlmeier-Degos' disease)

J Natl Med Assoc. 2005 Mar;97(3):421-5.


Malignant atrophic papulosis (MAP) is a rare and clinically distinctive vasculopathy. It is characterized by narrowing and occlusion of the lumen by intimal proliferation and thrombosis, which leads to ischemia and infarction in the involved organ systems. Its rarity and rapidly fatal course make the disease a difficult diagnostic and therapeutic challenge.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Abdominal Pain / etiology
  • Adult
  • Cerebral Infarction / etiology
  • Diarrhea / etiology
  • Humans
  • Male
  • Pleural Effusion / etiology
  • Recurrence
  • Skin Diseases, Papulosquamous / complications*
  • Skin Diseases, Vascular / complications*
  • Thromboangiitis Obliterans / complications*
  • Vomiting / etiology