摘要
Introduction: Aplasia cutis congenita is a rare congenital dermatosis of which type VI represents the Bart’s syndrome. The aim of this case is to describe the epidemiological, clinical, therapeutic and prognostic characteristics of this condition in a country with limited resources, for the improvement of prognosis and professional practice. Observation: This is a eutrophic newborn, born at term by vaginal delivery, who presented at birth with a unilateral absence of skin on the anteromedial aspect of the right leg starting from the knee and extending to the medial aspect of the right foot, with a dystrophy of the nail of the right big toe without any other visible physical malformation. The evolution was marked at D3 of life by the appearance of bullae on the right hand and elbow as well as on the posterior aspect of the neck, making epidermolysis bullosa suspect. The mother was 38 years old, 8<sup>th</sup> gesture, 7<sup>th</sup> pare with history of consanguinity and collodion baby. The association of a localized congenital absence of skin on the lower limbs, epidermolysis bullosa and a nail anomaly led to the diagnosis of congenital cutaneous aplasia of type VI of Frieden’s classification or Bart’s syndrome. The evolution was satisfactory on the 7<sup>th</sup> day of life with the beginning of scarring. The management was medical. The outcome was unfavorable with the appearance of sepsis and hemorrhage leading to death. Conclusion: Although rare, the clinical diagnosis of Bart’s syndrome is simple. However, the management is complex and the prognosis is reserved. To improve this prognosis, the treatment must guarantee excellent control of the infectious and hemorrhagic risks, an adhesion and good therapeutic compliance by the parents and a rigorous monitoring.
Introduction: Aplasia cutis congenita is a rare congenital dermatosis of which type VI represents the Bart’s syndrome. The aim of this case is to describe the epidemiological, clinical, therapeutic and prognostic characteristics of this condition in a country with limited resources, for the improvement of prognosis and professional practice. Observation: This is a eutrophic newborn, born at term by vaginal delivery, who presented at birth with a unilateral absence of skin on the anteromedial aspect of the right leg starting from the knee and extending to the medial aspect of the right foot, with a dystrophy of the nail of the right big toe without any other visible physical malformation. The evolution was marked at D3 of life by the appearance of bullae on the right hand and elbow as well as on the posterior aspect of the neck, making epidermolysis bullosa suspect. The mother was 38 years old, 8<sup>th</sup> gesture, 7<sup>th</sup> pare with history of consanguinity and collodion baby. The association of a localized congenital absence of skin on the lower limbs, epidermolysis bullosa and a nail anomaly led to the diagnosis of congenital cutaneous aplasia of type VI of Frieden’s classification or Bart’s syndrome. The evolution was satisfactory on the 7<sup>th</sup> day of life with the beginning of scarring. The management was medical. The outcome was unfavorable with the appearance of sepsis and hemorrhage leading to death. Conclusion: Although rare, the clinical diagnosis of Bart’s syndrome is simple. However, the management is complex and the prognosis is reserved. To improve this prognosis, the treatment must guarantee excellent control of the infectious and hemorrhagic risks, an adhesion and good therapeutic compliance by the parents and a rigorous monitoring.
