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Clin Rheumatol. 2006 Feb;25(1):88-91. Epub 2005 Oct 19.

Maffucci's syndrome: a case report.

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1
Rheumatology B Department, El Ayachi Hospital Rabat-Sale University Hospital, Sale, Morocco. faik_afaf@hotmail.com

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.

PMID:
16234991
DOI:
10.1007/s10067-005-1101-1
[Indexed for MEDLINE]
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