TITLE:
Bart’s Syndrome: A Neonatal Observation about a Case Report
AUTHORS:
Assetou Cissouma, Djibril Kassogue, Mala Sylla, Guedioma Dembélé, Soumaila Alama Traoré, Abdoulaye Kissima-Traoré, Dadé Ben Sidi Haidara, Mamadou Bernard Coulibaly, Madou Traore, Mady Niakaté
KEYWORDS:
Skin Aplasia, Congenital, Newborn, Skin Black
JOURNAL NAME:
Open Journal of Pediatrics,
Vol.11 No.3,
September
3,
2021
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.