Esophageal Stenosis as a Rare Mode of Revelation of Hereditary Epidermolysis Bullosa: Report of Three Cases

Chama ELMANJRA; Houda Tadili; Ibtissam Bounouar; Naima El Azzam; Oussama Nacir; Fatima Ezzahra Lairani; Maryam Aboudourib; Adil Ait Errami; Sofia Oubaha; Ouafa Hocar; Said Amal; Zouhour Samlani; Khadija Krati

doi:10.23958/ijirms/vol08-i12/1794

Open Access Publication ISSN: 2455-8737 |

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Case Report | Open Access

Vol. 8 No. 12 (2023) | Page No.: 535 - 540 | https://doi.org/10.23958/ijirms/vol08-i12/1794

Esophageal Stenosis as a Rare Mode of Revelation of Hereditary Epidermolysis Bullosa: Report of Three Cases

El Manjra Chama⁺⁻
Tadili Houda⁺⁻
Bounouar Ibtissam⁺⁻
El Azzam Naima⁺⁻
Nacir Oussama⁺⁻
Lairani Fatima Ezzahra⁺⁻
Aboudourib Maryam⁺⁻
Ait Errami Adil⁺⁻
Oubaha Sofia⁺⁻
Hocar Ouafa⁺⁻
Amal Said⁺⁻
Samlani Zouhour⁺⁻
Krati Khadija⁺⁻

Gastroenterology Department at Mohamed VI Universal Hospital of Marrakesh, Morocco.

Dermatology and Venerology Department at Mohamed VI Universal Hospital of Marrakesh, Morocco.

Gastroenterology Department at Mohamed VI Universal Hospital of Marrakesh, Morocco.

Dermatology and Venerology Department at Mohamed VI Universal Hospital of Marrakesh, Morocco.

Gastroenterology Department at Mohamed VI Universal Hospital of Marrakesh, Morocco.

Laboratory of Physiology, University Cadi Ayyad, Marrakesh, Morocco.

Dermatology and Venerology Department at Mohamed VI Universal Hospital of Marrakesh, Morocco.

Gastroenterology Department at Mohamed VI Universal Hospital of Marrakesh, Morocco.

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Published: 2023-12-15

Abstract

Dystrophic epidermolysis bullosa (DEB) is a heterogeneous group of rare genodermatoses with multiple variants and multi-organ involvement, including digestive system with esophageal stenosis. We describe in this paper 3 unusual cases of hereditary epidermolysis bullosa revealed by dysphagia. The first one is a 77-year-old man admitted with chronic dysphagia, cutaneous erosions and a complete loss of fingers and toenails. Gastroscopy with skin biopsies and barium swallow confirmed a hereditary epidermolysis bullosa. He was treated by pneumatic dilatation. The second case is a 60-year-old female admitted because of dysphagia. She was on good condition but had lingual erosions and anonychia affecting fingernails and toenails, with esophageal stenosis. The skin biopsy confirmed the DEB. She was treated successfully with pneumatic dilatation. The third case is a 15-year-old male who was transferred to our department for management of esophageal stenosis. The physical examination showed malnutrition and dehydration with bullous lesions and anonychia. The diagnostic of DEB was confirmed and he was treated by pneumatic dilatation. Dysphagia can rarely reveal an esophageal stenosis complicating a DEB, and can be treated successfully by pneumatic dilatation.

Keywords: Hereditary epidermolysis bullosa, Dermo-epidermal cohesion, Dysphagia, Esophageal stenosis, Pneumatic dilatation, Morocco

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How to Cite

ELMANJRA, C., Tadili, H., Bounouar, I., El Azzam, N., Nacir , O., Lairani, F. E., … Krati, K. (2023). Esophageal Stenosis as a Rare Mode of Revelation of Hereditary Epidermolysis Bullosa: Report of Three Cases. International Journal of Innovative Research in Medical Science, 8(12), 535–540. https://doi.org/10.23958/ijirms/vol08-i12/1794

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Copyright © 2023 El Manjra Chama Tadili Houda Bounouar Ibtissam El Azzam Naima Nacir Oussama Lairani Fatima Ezzahra Aboudourib Maryam Ait Errami Adil Oubaha Sofia Hocar Ouafa Amal Said Samlani Zouhour Krati Khadija this is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

This work is licensed under a Creative Commons Attribution 4.0 International License.