Otorhinolaryngological and esophageal manifestations of epidermolysis bullosa.
نویسندگان
چکیده
UNLABELLED Epidermolysis bullosa (EB) is a group of skin diseases with different clinical manifestations and varied inheritance patterns. Blisters may appear spontaneously or following minimal trauma to the skin or mucosa. AIM this paper aims to describe the otorhinolaryngological manifestations and esophageal complications related to EB, and the experience in treating patients with esophageal stenosis secondary to this disease. MATERIALS AND METHOD this descriptive study enrolled 60 patients with EB seen from June 1999 to December 2006 at the Head and Neck Surgery Service of X Hospital, a reference center for EB. RESULTS the patients' mean age was 14.5 years. Twenty-eight (46.6%) were females and 32 (53.4%) were males. Eight (13.4%) were diagnosed with epidermolysis bullosa simplex, while 51 (85%) had epidermolysis bullosa dystrophica; one (1.6%) patient had one acquired EB. Lips, mouth, tongue and ears were the most frequently involved sites (32 patients - 53.3%). Dysphagia was found in 28 patients (46.6%). After esophageal dilatation the symptoms subsided. CONCLUSION EB is a rare disease and patients must be sent for treatment at reference centers. Physicians treating patients for EB must be aware of the measures required to improve the quality of the treatment provided without putting the patients in harm's way.
منابع مشابه
FREE JEJUNAL GRAFT FOR TREATMENT OF ESOPHAGEAL STRICTURE DUE TO FAMILIAL EPIDERMOLYSIS BULLOSA
In this paper, two patients with severe dysphagia and esophageal stricture secondary to epidermolysis bullosa are presented and discussed. They are siblings of an affected family. Primary resection and anastomosis had been performed previously in both patients, but dysphagia had recurred. We treated these patients with free jejunal graft to the esophagus with excellent results. Both patient...
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ورودعنوان ژورنال:
- Brazilian journal of otorhinolaryngology
دوره 74 5 شماره
صفحات -
تاریخ انتشار 2008