AN ATYPICAL PEDIATRIC CASE OF MALIGNANT ATROPHIC PAPULOSIS (KOHLMEIER-DEGOS DISEASE)

A new case of malignant atrophic papulosis (Köhlmeier-Degos disease) is reported. Vascular symptoms began at 17 months of age with cerebral ischaemia and progressive involvement of fingers and toes with torpid ulcers and apical necrotic amputations. At 6 years of age he developed chronic intestinal ischaemia with malabsorption and a new cerebral attack; in spite of anti-aggregant therapy the disease progressed and he died 7 months after diagnosis from a third cerebral ictus. Since the typical skin lesions of the disease were absent, the diagnosis was made on the basis of a pathological pattern of an occluded biopsed artery. The elder brother presents clinical and instrumental vascular involvement without cutaneous lesions and could be slightly affected. © 1990 Springer-Verlag.