DOI: 10.36347/sjmcr.2023.v11i08.028

Pages: 1542-1546

Background: Bart syndrome is a rare neonatal condition that combines congenital skin aplasia and congenital bullous epidermolysis. The clinical diagnosis is straightforward, but its management is intricate, and the extensive forms pose a true therapeutic challenge. We report a case of a newborn diagnosed in the pediatric intensive care unit at the Military Hospital of Rabat. Case Description: A female newborn from a well-followed pregnancy, delivered via cesarean section due to a narrow pelvis. The Apgar score was 10/10, and the birth weight was 2600g. The mother had been diagnosed with hypothyroidism during pregnancy and was taking synthetic antithyroid medication. The infant was admitted to the neonatal unit at 2 hours of life due to multiple skin ulcers noted at birth. Clinical examination revealed bilateral and symmetrical cutaneous aplasia affecting almost the entirety of both lower limbs. The vascular network was clearly visible. Other parts of the skin showed areas of skin fragility and some flaccid blisters. A clinical diagnosis of Bart syndrome associated with bullous epidermolysis was made. Hydration and antibiotic coverage were initiated, leading to good clinical improvement. Discussion: Bart syndrome represents clinical features of aplasia cutis congenita associated with congenital epidermolysis bullosa suspected by areas of fragility and sometimes by blisters. All types of congenital epidermolysis bullosa can be associated with this syndrome. Clinical diagnosis is usually easy, but the therapeutic management is difficult, and the prognosis is poor.