DEGOS-DISEASE AND SPASTIC PARAPLEGIA

Malignant atrophic papulosis (Degos disease) is a rare disorder charac terized by a vasculopathy of unknown origin.1 The cutaneous manifestat ions comprise erythematous papules, which heal to leave scars with a p athognomonic central porcelain-white atrophic area and a peripheral te langiectatic rim. There is usually involvement of the gastrointestinal tract but other organ systems can also be affected, the central nervo us system being involved in 20% of cases.2 It is frequently fatal with in 2 or 3 years from onset of systemic involvement, the cause of death usually being intestinal perforation.3 Our patient is of interest as she has survived an unusually long time despite florid cutaneous and n eurological manifestations.