Proteus Syndrome

**Definition:** Hamartoneoplastic malformation syndrome of uncertain etiology characterized by partial GIGANTISM of the hands and/or feet, asymmetry of the limbs, plantar hyperplasia, hemangiomas (HEMANGIOMA), lipomas (LIPOMA), lymphangiomas (LYMPHANGIOMA), epidermal NEVI; MACROCEPHALY; cranial HYPEROSTOSIS, and long-bone overgrowth. Joseph Merrick, the so-called elephant man, apparently suffered from Proteus syndrome and not NEUROFIBROMATOSIS, a disorder with similar characteristics.

**Tree numbers:** - C04.445.435.500 - C04.651.435.500 - C05.116.099.750 - C05.660.585.620 - C16.131.077.740 - C16.131.621.585.620

**Synonyms:** - Elephant Man Disease