Degos disease and spastic paraplegia

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.