title: Proteus syndrome.
abstract: Proteus syndrome is a rare sporadic, hamartoneoplastic disorder of vascular, skeletal, and soft tissues that causes asymmetry of the skull, body, arms, and the legs. The name "Proteus" of the Greek god who had the ability to change his shape was coined to define the variety of deformities including partial gigantism of the hands or feet, asymmetry of the arms and legs, hypertrophy of long bones, plantar hyperplasia, haemangiomas, lipomas, varicosities, linear verrucous epidermal naevi, macrocephaly, and cranial hyperostoses. The basic defect seems to be the focal overgrowth of cellular elements in skin, bone, and other connective tissues. The variable features of the syndrome make differential diagnosis challenging for clinicians. The most important features are the hamartomatous disorders. The long-term prognosis is still not clear. As it is a hamartoneoplastic and incompletely delineated syndrome, the patients must be followed up because of the possible risk of neoplasms.
authors.affiliation: Plastic and Reconstructive Surgery Department, Ege University, Bornova, Izmir, Turkey. ubilkay@hotmail.com
authors.forename: Ufuk
authors.initials: U
authors.lastname: Bilkay
authors.forename: Cenk
authors.initials: C
authors.lastname: Tokat
authors.forename: Cuneyt
authors.initials: C
authors.lastname: Ozek
authors.forename: Hakan
authors.initials: H
authors.lastname: Gundogan
authors.forename: Ozgur
authors.initials: O
authors.lastname: Erdem
authors.forename: Tahir
authors.initials: T
authors.lastname: Gurler
authors.forename: Arman
authors.initials: A
authors.lastname: Cagdas
mesh_terms: D002675:Child, Preschool; D006801:Humans; D008297:Male; D016715:Proteus Syndrome