Otorhinolaryngological and esophageal manifestations of epidermolysis bullosa.

نویسندگان

  • Rodrigo Santana Fantauzzi
  • Mariana Oliveira Maia
  • Flávia Coelho Cunha
  • Rodrigo Vidal Simões
  • Denise Utsch Gonçalves
  • Amélio Ferreira Maia
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.

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عنوان ژورنال:
  • Brazilian journal of otorhinolaryngology

دوره 74 5  شماره 

صفحات  -

تاریخ انتشار 2008