Maffucci's syndrome: amesenchymal dysplasia and multiple tumour syndrome.
A case of Maffucci's syndrome with cutaneous, bony and neurological complications is reported. The patient had lymphangiomatosis at birth and developed multiple cutaneous haemangiomas and osteochondromas during childhood. She also developed multiple neurological defects, including cranial nerve palsies due to an intracranial osteochondroma. The occurence of mesodermal dysplasias and neoplasias in the Maffucci syndrome is emphasized, and it is suggested that there is a close relationship between this disorder and Ollier's disease (multiple enchondromatosis).
D000293:Adolescent; D000798:Angiomatosis / Q000150:complications*; D002812:Chondroma / Q000150:complications* / Q000175:diagnosis; D005260:Female; D006391:Hemangioma / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D009461:Neurologic Manifestations; D010009:Osteochondrodysplasias / Q000150:complications*; D012877:Skin Manifestations; D012888:Skull Neoplasms / Q000150:complications* / Q000175:diagnosis; D013577:Syndrome