Dyschondroplasia and Hemangiomata: Maffucci's Syndrome

Yonsei University College of Medicine, Seoul, Korea Maffucci's syndrome is characterized by dysccondroplasia with hemangiomata. Since Angelo Maffucci (1881) first described this entity, 105 cases and some more were reported in the literature till 1976. We recently observed a patient suffering from this disorder. The patient is 21 year-old girl, who has multiple hemangiomes on her right foot, right buttock, left wrist and enchondromas on both proximal phalanx, distal phalanx of both great toes and proximal, middle phalanx of right second toe. The disease started at age of 11 and these lesions grew rapidly last 3 years. The diagnosis was based on clinical, radiological and histological findings. Because of its rarity, we report it with brief review of literature.