Cutaneous Features of Crouzon Syndrome With Acanthosis Nigricans

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Cutaneous features of Crouzon syndrome with acanthosis nigricans.

IMPORTANCE Crouzon syndrome with acanthosis nigricans is a distinct disorder caused by a mutation in the FGFR3 gene, featuring craniosynostosis, characteristic facial features, and atypical and extensive acanthosis nigricans. Other cutaneous findings have not been thoroughly described. OBSERVATIONS We report 6 cases and summarize the existing literature with regard to the cutaneous manifestat...

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Crouzon syndrome in association with acanthosis nigricans

Crouzon syndrome is a rare inherited autosomal dominant syndrome characterized by craniosynostosis, midface hypoplasia. Acanthosis nigricans may be associated with Crouzon syndrome, but it differs from the classic crouzon syndrome. This is a report of a 30-year-old-woman who presented acanthosis nigricans coexist with crouzon syndrome.

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A recurrent mutation, ala391glu, in the transmembrane region of FGFR3 causes Crouzon syndrome and acanthosis nigricans.

Mutations in the fibroblast growth factor receptor 2 (FGFR2) gene have previously been identified in Crouzon syndrome, an autosomal dominant condition involving premature fusion of the cranial sutures. Several different missense and other mutations have been identified in Crouzon syndrome patients, clustering around the third immunoglobulin-like domain. We report here the identification of a mu...

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Surgical treatment for eyelid deformity in Crouzon syndrome associated with acanthosis nigricans: case report.

The combination of Crouzon syndrome with acanthosis nigricans is unusual. The incidence of this combination is still uncertain, and there are only 30 cases reported in the medical literature. Crouzon syndrome is caused by mutation of the gene FGFR3 (fibroblast growth factor receptor 3). In this gene, other mutations can cause a kind of nanism that is lethal during the first weeks of life. Recen...

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Malignant acanthosis nigricans, florid cutaneous papillomatosis and tripe palms syndrome associated with gastric adenocarcinoma

Malignant acanthosis nigricans is a rare paraneoplastic skin syndrome mostly associated with gastric adenocarcinoma. Florid cutaneous papillomatosis and tripe palms syndrome are considered to be abortive clinical variants of acanthosis nigricans. Clinical manifestations include pruritic, hyperkeratotic and hyperpigmented plaques with a subsequent formation of velvety papillomas in the involved ...

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ژورنال

عنوان ژورنال: JAMA Dermatology

سال: 2013

ISSN: 2168-6068

DOI: 10.1001/jamadermatol.2013.3019