Proteus Syndrome

• Sporadic, mosaic mutation in PTEN
• Named after the Greek god, Proteus, who could change his shape at will (due to dramatic variation in manifestations of syndrome)
• Cutaneous findings: hyperkeratotic epidermal nevi, palmoplantar cerebriform connective tissue nevi, capillary malformation, hemangiomas, lipomas
• Systemic findings: asymmetric growth with partial gigantism of hands/feet, hyperostoses of epiphyses and skull (especially external auditory canal), bilateral ovarian cystadenomas