Proteus Syndrome of the Hand

Fraiture, Anne Laure; Ihou, E David; Pierard, Gérald

doi:10.1159/000063375

Cited by 5 publications

(1 citation statement)

References 17 publications

(21 reference statements)

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“…There are many reports in the literature of macrodactily with skin hypertrophy, affecting fingers or toes [5][6][7][8][9] some other cases were published as isolated macrodactily, probably before the description of the Proteus syndrome [10][11] . This minimal acral variant should be included in the spectrum of the Proteus Syndrome and should be recognized by dermatologists.…”

Section: Discussionmentioning

confidence: 99%

Macrodactyly with skin hypertrophy: a minimal form of the Proteus syndrome

Almeida

Fiss

Happle

2011

An. Bras. Dermatol.

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The Proteus syndrome was described 1983 . It has asymmetric gigantism of the limbs, verrucous epidermal naevi, cerebriform enlargement of the plantar region, vascular malformations and neoplasms, as lipomas. It received this denomination after Proteus from the Greek mythology, who had the ability to change his form . A 15 year-old boy, reported a congenital hypertrophy with syndactily of the second and third right fingers . The second case is a 35 year-old man, who reported that since birth the second right toe was bigger than the other toes, skin hypertrophy was also observed. These cases document a localized form if the Proteus syndrome, which may widen the spectrum of its variability. Keywords: Hypertrophy; Hand deformities; Foot deformities Resumo: A síndorme de Proteus foi descrita por Wiedemann em 1983. Ela é caracterizada por gigantismo assimétrico dos membros, nevos epidérmicos verrucosos, hipertrofia cerebriforme da região plantar, neoformações vasculares e neoplasias, como lipomas. Essa polimórfica enfermidade recebeu essa denominação segundo a figura da mitologia grega, a qual tinha como característica a habilidade de mudar de forma, para evitar sua captura. Examinou-se um menino de 15 anos, o qual referiu hipertrofia e sindactilia do segundo e terceiro quirodáctilos direitos e um homem de 35 anos, que referiu hipertrofia congênita do segundo pododáctilo direito. Havia hipertrofia da pele em ambos os casos. Esses dois pacientes documentam formas localizadas dessa síndrome, ampliando seu espectro clínico.

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Section: Discussionmentioning

confidence: 99%