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Войти в исследования La Tunisie medicale · 2026
[Inherited epidermolysis bullosa in Dakar : Descriptive cross-sectional study of 36 cases].
Аннотация на английскомСтатьяНазвание и аннотация: Перевод в очереди
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Ndiaye Diop Mame Tene, Diop Khadim, Seck Birame, Ba Khadija, Fall Fatou Diassé, Niang Babacar, Diallo Moussa, Ndiaye Ousmane, Ndiaye Maodo, Ly Fatimata, Niang Suzanne Oumou
Аннотация
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.
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.