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ULTIMO FASCICOLOGIORNALE ITALIANO DI DERMATOLOGIA E VENEREOLOGIA

Rivista di Dermatologia e Malattie Sessualmente Trasmesse


Official Journal of the Italian Society of Dermatology and Sexually Transmitted Diseases
Indexed/Abstracted in: EMBASE, PubMed/MEDLINE, Science Citation Index Expanded (SciSearch), Scopus
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Giornale Italiano di Dermatologia e Venereologia 2016 Jul 8

lingua: Inglese

Kindler syndrome: a case of two Iranian sisters

Saeed KARGAR 1, Seyed M. SHIRYAZDI 1, Hossein NEAMATZADEH 2, V. RAMAZANI 3

1 Department of Surgery, Shahid Sadoughi Hospital, Shahid Sadoughi University of Medical Sciences and Health Services, Yazd, Iran; 2 Msc of Human Molecular Genetics, Shahid Sadoughi University of Medical Sciences and Health Services, Yazd, Iran; 3 Islamic Azad University Ashkzar Branch, Ave-Sina Blvd, Shahid Ghandi Blvd, Ashkzar, Yazd, Iran


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Kindler syndrome is a rare autosomal recessive condition, characterized by multiple skin and mucosal abnormalities. Among the latter, esophageal involvement is an infrequent manifestation which may be completely asymptomatic or complicate with dysphagia. We report two sisters presenting with cutaneous features and severe dysphagia. Endoscopic examination showed that the patients were affected with a rare condition named esophageal web. Both patients showed much improvement after balloon dilation. Clinicians should be aware of the potential disease complications and balloon dilation approach should be considered as primary therapy in the Kindler syndrome patients with esophageal web.

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