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 t...

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Main Authors: Ritha de Cássia Capelato Rocha (Author), Mariani Paulino Soriano Estrella (Author), Danielle Mechereffe do Amaral (Author), Angela Marques Barbosa (Author), Marilda Aparecida Milanez Morgado de Abreu (Author)
Format: Book
Published: Sociedade Brasileira de Dermatologia, 2017-10-01T00:00:00Z.
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Summary: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.
Item Description:0365-0596
10.1590/abd1806-4841.20174496