C3213Level 6
Maffucci Syndrome
**Semantic type:** Disease or Syndrome
**Definition:** A rare non-inherited disorder primarily affecting the skin and skeletal system. It is classified as a mesodermal dysplasia. Clinical signs appear within the first decade and are characterized by multiple soft tissue hemiangiomas and enchondromas leading to skeletal deformities. Clinical course is progressive with variable development of associated malignancies.
**Synonyms:** - Chondrodysplasia with Hemangioma - Chondroplasia Angiomatosis - Dyschondroplasia and Cavernous Hemangioma - Enchondromatosis with Hemangiomata - Hemangiomata with Dyschondroplasia - Maffucci's Anomalad
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