Maffucci's syndrome: a mesenchymal dysplasia and multiple tumour syndrome

Abstract

A case of Maffucci''s syndrome with cutaneous, bony and neurological complications is reported. The patient had lymphangiomatosis at birth and developed multiple cutaneous hemangiomas and osteochondromas during childhood. She also developed multiple neurological effects, including cranial nerve palsies due to an intracranial osteochondroma. The occurrence 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).