Patients are normal at birth and the syndrome manifests during childhood and puberty. The enchondromas affect the extremities and their distribution is asymmetrical. The most common sites of enchondromas are the metacarpal bones and phalanges of the hands. The feet are less commonly afflicted. Disfigurations of the extremities are a result. Pathological fractures can arise in affected metaphyses and diaphyses of the long bones and are common (26%). The risk for sarcomatous degeneration of enchondromas, hemangiomas, or lymphangiomas is 15–30% in the setting of<unk> .<unk>  is associated with a higher risk of CNS, pancreatic, and ovarian malignancies. Multiple enchondromas may present in three disorders: Ollier disease,<unk> , and metachondromatosis. It is important to make the distinction between these diseases, particularly Ollier disease and<unk> . Ollier disease is more common than<unk> , and presents with multiple enchondromas often in a unilateral distribution. However, hemangiomas and lymphangiomas are not seen in Ollier disease. Metachondromatosis demonstrates autosomal-dominant transmission and presents with both multiple osteochondromas and enchondromas. It is associated with multiple cavernous hemangioma and phlebolith. Lymphangiomas may also be apparent.
Maffucci syndrome
Jalili syndrome
Ogden syndrome
Heerfordt syndrome
BENTA disease
Ehlers–Danlos syndromes
Senior–Løken syndrome
Jaffe–Campanacci syndrome