A 39 year old man presented with recurrent episodes of leukocytoclastic vasculitis and a large ulcer at the distal part of the stump of his right leg. Since birth the patient showed a clinical picture of pseudoainhum as part of the amniotic band syndrome (Simonart syndrome). Radiologic examination of the right foot showed only an hypoplastic talus and calcaneus which had been designated as a "stump":, which have been detected by x-ray. On both upper limbs there were missing phalanges, syndactyly and some constrictive fibrotic bands. Since the ulcer worsened with therapy, the patient underwent the amputation of the stump just below under the knee. Since then no further vasculitic lesions have been observed. We consider this case remarkable both because pseudoainhum is so rare and because of these equally unusual complications.
