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In 1966, Bart et al 1 described 20 members of a family with CAS affecting the lower extremities, blistering of the skin and mucous membranes, and nail abnormalities. This association of EB and CAS came to be known as Bart's syndrome (BS). At the time of the first report, the subtypes of EB were not classified because ultrastructural and immunochemical studies were not available. Years later, Zelickson et al 2 performed ultrastructural studies on the initial cases, demonstrating findings of dominant dystrophic epidermolysis

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Bart’s Syndrome: A Neonatal Observation about a Case Report

Cissouma¹,

Kassogué²,

Sylla³

et al. 2021

OJPed

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Introduction: Bart's syndrome is a rare neonatal pathology combining congenital skin aplasia affecting the extremities and congenital epidermolysis bullosa, exceptionally described on black skin. Observation: A 2-day-old male newborn was referred for multiple ulcerations of the limbs observed at birth. The clinical examination found an absence of bilateral and symmetrical skin occupying almost all of the two lower limbs with some flaccid bubbles. The vascular network was clearly visible. The rest of the skin coating was normal. The diagnosis of Bart syndrome in connection with epidermolysis bullosa was evoked clinically and despite pediatric and dermatological management, the evolution was rapidly fatal by severe sepsis. Discussion: Bart syndrome corresponds to a clinical picture of congenital skin aplasia associated with congenital epidermolysis bullosa suspected by areas of fragility and sometimes bubbles. All types of congenital epidermolysis bullosa may be associated with this syndrome. The clinical diagnosis is generally easy but the therapeutic management is difficult and the prognosis reserved. Conclusion: Bart syndrome is a curious congenital association of well-defined skin symptoms, the etiopathogeny of which still remains poorly elucidated, hence the difficulty of establishing an antenatal diagnostic strategy or giving appropriate genetic advice.

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Cited by 3 publications

References 5 publications

Syndrome de Bart : une nouvelle observation néonatale

Syndrome de Bart : une nouvelle observation néonatale

Epidermolysis bullosa with congenital absence of skin: Review of the literature

Bart’s Syndrome: A Neonatal Observation about a Case Report

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