Inherited epidermolysis bullosa in Dakar : Descriptive cross-sectional study of 36 cases
DOI:
https://doi.org/10.62438/tunismed.v104i04.6176Keywords:
Inherited epidermolysis bullosa, dystrophic inherited epidermolysis bullosa, infection, cancer, DakarAbstract
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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