Inherited epidermolysis bullosa in Dakar : Descriptive cross-sectional study of 36 cases

Authors

  • Mame Tene Ndiaye Diop Department of Dermatology, Department of Paediatrics, National Children’s Hospital Albert Royer, Department of Dermatology, PO Box 25755 Dakar, Faculty of Medicine, Pharmacy and Dentistry, Cheikh Anta Diop University of Dakar, Fann, Senegal
  • Khadim Diop Department of Dermatology, Albert Royer National Children’s Hospital, Department of Dermatology, Faculty of Medicine, Pharmacy and Dentistry, Cheikh Anta Diop University of Dakar, PO Box 25755 Dakar, Fann, Senegal
  • Birame Seck Department of Dermatology, Saint-Louis, Gaston Berger University of Saint-Louis, Senegal
  • Khadija Ba Department of Dermatology, Albert Royer National Children’s Hospital, Department of Dermatology, Faculty of Medicine, Pharmacy and Dentistry, Cheikh Anta Diop University of Dakar, PO Box 25755 Dakar, Fann, Senegal
  • Fatou Diassé Fall Department of Dermatology, Albert Royer National Children’s Hospital, Department of Dermatology, Faculty of Medicine, Pharmacy and Dentistry, Cheikh Anta Diop University of Dakar, PO Box 25755 Dakar, Fann, Senegal
  • Babacar Niang Paediatrics Department, Albert Royer National Children’s Hospital, Faculty of Medicine, Pharmacy and Dentistry, Cheikh Anta Diop University of Dakar, PO Box 25755 Dakar, Fann, Senegal
  • Moussa Diallo Department of Dermatopathology, PO Box 25755 Dakar, Cheikh Anta Diop University of Dakar, Fann, Senegal
  • Ousmane Ndiaye Paediatrics Department, Albert Royer National Children’s Hospital, Faculty of Medicine, Pharmacy and Dentistry, Cheikh Anta Diop University of Dakar, PO Box 25755 Dakar, Fann, Senega
  • Maodo Ndiaye Department of Dermatology, Albert Royer National Children’s Hospital, Department of Dermatology, Faculty of Medicine, Pharmacy and Dentistry, Cheikh Anta Diop University of Dakar, PO Box 25755 Dakar, Fann, Senegal
  • Fatimata Ly Department of Dermatology, Albert Royer National Children’s Hospital, Department of Dermatology, Faculty of Medicine, Pharmacy and Dentistry, Cheikh Anta Diop University of Dakar, PO Box 25755 Dakar, Fann, Senegal
  • Suzanne Oumou Niang Department of Dermatology, Albert Royer National Children’s Hospital, Department of Dermatology, Faculty of Medicine, Pharmacy and Dentistry, Cheikh Anta Diop University of Dakar, PO Box 25755 Dakar, Fann, Senegal

DOI:

https://doi.org/10.62438/tunismed.v104i04.6176

Keywords:

Inherited epidermolysis bullosa, dystrophic inherited epidermolysis bullosa, infection, cancer, Dakar

Abstract

Introduction: The aim of this study was to determine the frequency, clinical forms and complications of Inherited epidermolysis bullosa in Dakar.
Methodology: we conducted a descriptive cross-sectional study with data collection from medical records of patients followed up for Inherited
epidermolysis bullosa in the dermatology department of the Albert Royer Children's Hospital over an 11-year period.
Results: 36 patients were registered, representing a hospital frequency of 0.16% and a sex ratio of 0.6. Clinical forms were: dystrophic in 44%,
simple in 33%, junctional in 17%, associated with congenital cutaneous aplasia in 6% (Bart's syndrome), Kindler's syndrome in 6%. Complications
occurred in 39% of cases, including infections in 28%, hemodynamic disorders in 17% and digestive complications in 11%. Death was recorded in
11% as a result of infectious and anemia.
Conclusion: We report a series of 36 cases of inherited epidermolysis bullosa, representing a hospital frequency of 0.16%. The most frequent
clinical form is dystrophic. Complications are mainly infectious and hemodynamic. We emphasize the importance of follow-up in these patients,
given the risk of skin cancer associated with the long-term prognosis of scars in dystrophic forms.

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2026-04-30

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