P 83 : Association d’une aplasie cutanée congénitale et d’une épidermolyse bulleuse congénitale (Syndrome de BART) : une observation inhabituelle sur peau noire

Ahogo, K.C.; Bamba, V.; Kaloga, M.; Gbery, I.P.; Allou, A.S.; Cissé, L.; Azagoh, Kouadio Richard; Kassi, K.; Kouamé, K.; Kouassi, K.A.; Ecra, E.; Kouassi, Y.M.; Kourouma, S.; Yobouet, P.; Kanga, J. M.

doi:10.1016/s0151-9638(16)30258-7

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2022

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“…In sub‐Saharan Africa, we have identified five cases of ACC affecting the lower limbs, including two cases of ACC type 6 or Bart's syndrome, respectively, in Nigeria and Côte d'Ivoire 6 , 7 and three cases of typical ACC type 7 in Nigeria. 8 , 9 …”

Section: Introductionmentioning

confidence: 99%

Bart's syndrome associated with a disorder of sexual differentiation: An atypical presentation in a Cameroonian newborn

Sigha

Betoko

Nkoro

et al. 2022

Clinical Case Reports

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Bart's syndrome consists of congenital aplasia of the skin affecting only the lower limbs, associated with bullae over the skin and/or mucous membranes, as well as a nail anomaly. It is an extremely rare genetic disorder, which can be associated with other birth defects. We report the case of a newborn baby admitted at day 0 of life in the neonatal department, for multifocal skin detachment predominantly at the lower limbs. In addition, examination of the external genitalia revealed a clitoridomegaly genital bud measuring 14 mm, scrotalized and unfused genital bulges with the presence of 2 orifices. No gonad was palpated. The clinical diagnosis of Bart's syndrome associated with a disorder of sexual differentiation was retained. We hereby report the first case of Bart's syndrome described in Cameroon in association with a disorder of sexual differentiation.

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Section: Introductionmentioning

confidence: 99%