Otorhinolaryngological and esophageal manifestations of epidermolysis bullosa.
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.


OBJECTIVE
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.


METHODS
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.


CONCLUSIONS
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.
Fantauzzi
Rodrigo Santana
RS
Otorhinolaryngologist, Member of the Clinical Team at Hospital Municipal de Contagem.
Maia
Mariana Oliveira
MO
MD, Resident at the ENT Service at Hospital das Clínicas da UFMG.
Cunha
Flávia Coelho
FC
Otorhinolaryngologist, Member of the Clinical Team at Hospital Júlia Kubitschek.
Simões
Rodrigo Vidal
RV
Otorhinolaryngologist, Member of the Clinical Team at Hospital Militar do Estado de Minas Gerais.
Gonçalves
Denise Utsch
DU
PhD, Adjunct Professor of the Department of Ophthalmology, Otorhinolaryngology, and Speech and Hearing Therapy at the UFMG Medical School, Otorhinolaryngologist and Full Advisor at the Graduate Program on Infectology and Tropical Medicine at the UFMG Medical School.
Maia
Amélio Ferreira
AF
Otorhinolaryngologist, Head and Neck Surgeon, Coordinator of the Otorhinolaryngology and Head and Neck Surgery Clinic at Hospital Felício Rocho. Hospital Felício Rocho.
D000293:Adolescent; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D003680:Deglutition Disorders / Q000209:etiology; D004820:Epidermolysis Bullosa / Q000150:complications* / Q000628:therapy; D016108:Epidermolysis Bullosa Dystrophica / Q000150:complications / Q000628:therapy; D016110:Epidermolysis Bullosa Simplex / Q000150:complications / Q000628:therapy; D004935:Esophageal Diseases / Q000209:etiology* / Q000628:therapy; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010038:Otorhinolaryngologic Diseases / Q000209:etiology*; D055815:Young Adult