Maffucci syndrome is a rare disorder that mainly involves bones and skin. It causes non malignant cartilage tumors (enchondromas), deformities in the skeletal system, and hemangiomas (abnormally structured blood vessels, in skin and other organs) .As it was first observed by Maffucci in 1881, it was named as Maffucci Syndrome. Maffucci syndrome is different from various disorders of enchondromas (abnormal cysts of cartilage in bone marrow) with characteristic red/purplish growth/s on the skin, abnormal tangle of blood vessels (hemangiomas).
- Dyschondrodysplasia and hemangiomas
- Hemangiomatosis Chondrodystrophica
- Kast Syndrome
- Angioma and several Endochondromas
- Chondrodysplasia with hemangiomas
- Chondroplasia angiomatosis
- Cavernous hemangiomas and dyschondroplasia
- Enchondromatosis with hemangiomata
Age: Maffucci syndrome lesions are generally seen first at 4-5 years of age.
Incidence: Maffucci syndrome is very rare. Since first described in 1881, less than 200 cases reported all over the world.
There is no racial susceptibility or sexual variation is observed till date, nevertheless it cannot be ruled out as a result of limited number of cases. It is observed that around 25% of cases are congenital.