Degos disease and spastic paraplegia.

[malignant atrophic papulosis]

Malignant atrophic papulosis (Degos disease ) is a rare disorder characterized by a vasculopathy of unknown origin . The cutaneous manifestations comprise erythematous papules , which heal to leave scars with a pathognomonic central porcelain-white atrophic area and a peripheral telangiectatic rim . There is usually involvement of the gastrointestinal tract but other organ systems can also be affected , the central nervous system being involved in 20 % of cases . It is frequently fatal within 2 or 3 years from onset of systemic involvement , the cause of death usually being intestinal perforation . Our patient is of interest as she has survived an unusually long time despite florid cutaneous and neurological manifestations .