Proteus syndrome: A case report of a hamartomatous syndrome with severe mandibular hemihypertrophy

Proteus syndrome is a rare congenital hamartomatous malformation that is characterized by a wide range of deformities, including craniofacial deformities. Skin and skeletal developmental malformations are common and may assume tremendous proportions. The syndrome is often mistaken for other, more co...

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Veröffentlicht in:Oral surgery, oral medicine, oral pathology, oral radiology and endodontics oral medicine, oral pathology, oral radiology and endodontics, 1998, Vol.85 (1), p.82-85
Hauptverfasser: Pinto, Pascal X, Beale, Victoria, Paterson, Anthony W
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Beale, Victoria
Paterson, Anthony W
description Proteus syndrome is a rare congenital hamartomatous malformation that is characterized by a wide range of deformities, including craniofacial deformities. Skin and skeletal developmental malformations are common and may assume tremendous proportions. The syndrome is often mistaken for other, more commonly recognized conditions, including neurofibromatosis. The soft-tissue masses in Proteus syndrome are not nerve tumors but are usually hamartomatous proliferations. The case report describes its varied manifestations, which include significant cranifoacial dysmorphism, and discusses the differential diagnosis and management.
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subjects Adult
Biological and medical sciences
Complex syndromes
Dentistry
Facial Asymmetry - etiology
Female
Humans
Mandible - abnormalities
Medical genetics
Medical sciences
Proteus Syndrome - complications
Proteus Syndrome - pathology
title Proteus syndrome: A case report of a hamartomatous syndrome with severe mandibular hemihypertrophy
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