Abstract
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.
Highlights
IntroductionBart syndrome is a rare neonatal pathology combining congenital skin aplasia affecting the extremities and exceptionally described congenital epidermolysis bullosa on black skin, as well as ungeal deformities
Bart’s syndrome is a rare neonatal pathology combining congenital skin aplasia affecting the extremities and congenital epidermolysis bullosa, exceptionally described on black skin
Bart syndrome corresponds to a clinical picture of congenital skin aplasia associated with congenital epidermolysis bullosa suspected by areas of fragility and sometimes bubbles
Summary
Bart syndrome is a rare neonatal pathology combining congenital skin aplasia affecting the extremities and exceptionally described congenital epidermolysis bullosa on black skin, as well as ungeal deformities. Clinical diagnosis is easy but its management is delicate and the extended forms represent a real therapeutic challenge. It was first described in a family by Bart et al in 1966 [1]. About twenty forms of congenital and hereditary epidermolysis bullosa are observed and are classified into three groups according to the level where cleavage occurs: in the dermorepidermal junction zone: these are the simple and non-scarring inepidermal forms, at the level of the pars lucida: these are the junctional forms and under the basal lama: these are the dystrophic forms. We report an observation of a neonatal Bart syndrome on black skin, and propose a review of the literature
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
