Kindler syndrome: the case of two Iranian sisters.

Abstract

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 complicated by dysphagia. We report the case of two sisters presenting with cutaneous features and severe dysphagia. Endoscopic examination showed that the patients were affected by a rare condition named "esophageal web". Both patients showed significant improvement after balloon dilation. Clinicians should be aware of the potential complications of this disease, and the approach by balloon dilation should be considered as primary therapy in Kindler syndrome patients with esophageal web.

• Authors: Saeed Kargar, Seyed M Shiryazdi, Hossein Neamatzadeh, Vahid Ramazani
• Journal: Giornale italiano di dermatologia e venereologia : organo ufficiale, Societa italiana di dermatologia e sifilografia (G Ital Dermatol Venereol) Vol. 153 Issue 1 Pg. 111-114 (Feb 2018) ISSN: 1827-1820 [Electronic] Italy
• PMID: 27391311 (Publication Type: Case Reports, Journal Article)

Topics

• Adult
• Blister (diagnosis, physiopathology)
• Deglutition Disorders (etiology)
• Endoscopy (methods)
• Epidermolysis Bullosa (diagnosis, physiopathology)
• Esophagus (pathology)
• Female
• Humans
• Iran
• Periodontal Diseases (diagnosis, physiopathology)
• Photosensitivity Disorders (diagnosis, physiopathology)
• Siblings
• Young Adult