Proteus syndrome

• Autor(a) principal: Rocha,Ritha de Cássia Capelato
• Data de Publicação: 2017
• Outros Autores: Estrella,Mariani Paulino Soriano, Amaral,Danielle Mechereffe do, Barbosa,Angela Marques, Abreu,Marilda Aparecida Milanez Morgado de
• Tipo de documento: Relatório
• Idioma: eng
• Título da fonte: Anais brasileiros de dermatologia (Online)
• Texto Completo: http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017000500717
• topic: Congenital abnormalities; Genetics; Hyperplasia; Mosaicism; Neoplasms; Proteus; Syndrome

Abstract: Proteus syndrome is a rare syndrome characterized by disproportionate overgrowth of limbs, multiple hamartomas, and vascular malformations. The cerebriform connective tissue nevi, also called cerebriform plantar hyperplasia, are present in most patients, and is the main characteristic of the syndrome. If present, even alone, they can be considered as a pathognomonic sign. This article reports a classic case of Proteus syndrome in a 2-year-old male patient who began to show a discrete asymmetry of the right hemibody in relation to the left one after birth, which increased over the months. He also showed cerebriform plantar hyperplasia and Port-wine stains, among other alterations.