Otorhinolaryngological and esophageal manifestations of epidermolysis bullosa
Summary: 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...
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| Format: | Article |
| Language: | English |
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Elsevier
2008-09-01
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| Series: | Brazilian Journal of Otorhinolaryngology |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S1808869415313732 |
| _version_ | 1819179129269911552 |
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| author | Rodrigo Santana Fantauzzi Mariana Oliveira Maia Flávia Coelho Cunha Rodrigo Vidal Simões Denise Utsch Gonçalves Amélio Ferreira Maia |
| author_facet | Rodrigo Santana Fantauzzi Mariana Oliveira Maia Flávia Coelho Cunha Rodrigo Vidal Simões Denise Utsch Gonçalves Amélio Ferreira Maia |
| author_sort | Rodrigo Santana Fantauzzi |
| collection | DOAJ |
| description | Summary: 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. Keywords: epidermolysis bullosa, esophagus, manifestations, otorhinolaryngology, treatment |
| first_indexed | 2024-12-22T21:53:32Z |
| format | Article |
| id | doaj.art-9be444ac02b248dfab6a86570e42708c |
| institution | Directory Open Access Journal |
| issn | 1808-8694 |
| language | English |
| last_indexed | 2024-12-22T21:53:32Z |
| publishDate | 2008-09-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Brazilian Journal of Otorhinolaryngology |
| spelling | doaj.art-9be444ac02b248dfab6a86570e42708c2022-12-21T18:11:17ZengElsevierBrazilian Journal of Otorhinolaryngology1808-86942008-09-01745657661Otorhinolaryngological and esophageal manifestations of epidermolysis bullosaRodrigo Santana Fantauzzi0Mariana Oliveira Maia1Flávia Coelho Cunha2Rodrigo Vidal Simões3Denise Utsch Gonçalves4Amélio Ferreira Maia5Otorhinolaryngologist, Member of the Clinical Team at Hospital Municipal de ContagemMD, Resident at the ENT Service at Hospital das Clínicas da UFMG; Send correspondence to: Clínica de Otorrinolaringologia e Cirurgia de Cabeça e Pescoço, Hospital Felício Rocho - Avenida do Contorno 9530 3º andar Prado Belo Horizonte MG 30110-934. Phone number: +55 31 3292-8128Otorhinolaryngologist, Member of the Clinical Team at Hospital Júlia KubitschekOtorhinolaryngologist, Member of the Clinical Team at Hospital Militar do Estado de Minas GeraisPhD, 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 SchoolOtorhinolaryngologist, Head and Neck Surgeon, Coordinator of the Otorhinolaryngology and Head and Neck Surgery Clinic at Hospital Felício Rocho. Hospital Felício RochoSummary: 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. Keywords: epidermolysis bullosa, esophagus, manifestations, otorhinolaryngology, treatmenthttp://www.sciencedirect.com/science/article/pii/S1808869415313732 |
| spellingShingle | Rodrigo Santana Fantauzzi Mariana Oliveira Maia Flávia Coelho Cunha Rodrigo Vidal Simões Denise Utsch Gonçalves Amélio Ferreira Maia Otorhinolaryngological and esophageal manifestations of epidermolysis bullosa Brazilian Journal of Otorhinolaryngology |
| title | Otorhinolaryngological and esophageal manifestations of epidermolysis bullosa |
| title_full | Otorhinolaryngological and esophageal manifestations of epidermolysis bullosa |
| title_fullStr | Otorhinolaryngological and esophageal manifestations of epidermolysis bullosa |
| title_full_unstemmed | Otorhinolaryngological and esophageal manifestations of epidermolysis bullosa |
| title_short | Otorhinolaryngological and esophageal manifestations of epidermolysis bullosa |
| title_sort | otorhinolaryngological and esophageal manifestations of epidermolysis bullosa |
| url | http://www.sciencedirect.com/science/article/pii/S1808869415313732 |
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