title: Maffucci's syndrome: a case report.
abstract: Maffucci's syndrome is a congenital nonhereditary syndrome characterized by a combination of enchondromatosis and hemangiomatosis. It is associated with an increased risk of malignancy. We report a case of a 24-year-old woman who developed severe bone distortion due to generalized enchondromas since the age of 2 years. Physical examination showed a marked shortening of both upper and lower limbs and cutaneous hemangioma in the legs. Radiological study showed symmetric well-circumscribed radiolucent cystic lesions in the hands and severe metaphysical irregularity and a shortening of all long bones. The biopsy of the third right metacarpal showed enchondroma tissue. Histological examination of the skin specimen showed a hemangioendothelioma. The diagnosis of Maffucci's syndrome was established. Abdominal ultrasound, mammography, cerebral computed tomography (CT) scan, and cervical CT were normal.
authors.affiliation: Rheumatology B Department, El Ayachi Hospital Rabat-Sale University Hospital, Sale, Morocco. faik_afaf@hotmail.com
authors.forename: Afaf
authors.initials: A
authors.lastname: Faik
authors.forename: Fadoua
authors.initials: F
authors.lastname: Allali
authors.forename: Selma
authors.initials: S
authors.lastname: El Hassani
authors.forename: Najia
authors.initials: N
authors.lastname: Hajjaj-Hassouni
mesh_terms: D000328:Adult; D004687:Enchondromatosis / Q000000981:diagnostic imaging / Q000473:pathology*; D005260:Female; D006390:Hemangioendothelioma / Q000473:pathology; D006801:Humans; D011859:Radiography; D012878:Skin Neoplasms / Q000473:pathology