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

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.