TITLE:
Type VI Aplasia Cutis Congenita: About a Case Report at University Teaching Hospital of Bouaké
AUTHORS:
Yao Kouassi Christian, Yeboua Yao Kossonou Roland, Yenan John Patrick, Akanji Iburaima Alamun, Adou Leioh Romeo, Sahi Gnantin Josette Landryse, Amani Ehi Alexise Eleonore, Avi-Siallou Christelle Honorine, Aka-Tanoh Koko Aude Hélène, Asse Kouadio Vincent
KEYWORDS:
Aplasia Cutis Congenita, Bart’s Syndrome, Newborn, Prognosis, Côte d’Ivoire
JOURNAL NAME:
Open Journal of Pediatrics,
Vol.13 No.1,
January
29,
2023
ABSTRACT: 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, 8th gesture, 7th 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 7th 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.