Effective treatment of malignant atrophic papulosis (Köhlmeier-Degos disease) with treprostinil--early experience.

Background: Malignant atrophic papulosis (Köhlmeier-Degos disease; MAP) is an uncommon endotheliopathy with pathological findings similar to the vascular lesions of systemic sclerosis. These two disorders can overlap. When associated with visceral lesions, MAP has been considered almost universally and rapidly fatal.