The mature epidermis is a stratified epithelial tissue composed
predominantly of keratinocytes(4). A syndrome, as related to genetics,
is a group of traits or Conditions that tend to occur together and
Characterize a recognizable disease. Some syndromes have a genetic
Cause.
Bart syndrome is an unusual condition that combines features from two
distinct disorders, namely aplasia cutis congenita (ACC) and
epidermolysis bullosa (EB)(5). ACC, a rare congenital anomaly
characterized by the absence of skin, was initially documented in 1767
by Cordon(5). Frieden’s classification system categorizes ACC into nine
groups based on location and associated abnormalities(5).
Inherited EB is a group of genetically transmitted Skin disorders.
Ep can be part of other syndromes(6). It can be marked by skin and
mucous membrane fragility, resulting in blisters with minimal trauma(7).
The clinical diagnosis of aplasia cutis congenital, specifically Bart
syndrome, hinges primarily on identifying classical cutaneous
manifestations, with the extent of involvement contingent upon the mode
of inheritance(8).
In alignment with Omran et al. and Sharif, our case also exhibited skin
lesions and ear malformation(3). In this case, we have seen standard
Apgar scores like Sharif et al. (3). It could be better to have a skin
biopsy and Brain MRI. To achieve a precise postnatal classification of
inherited Epidermolysis Bullosa (EB), it is imperative to employ skin
biopsy as a fundamental diagnostic tool(8). This biopsy specimen should
undergo comprehensive examination, incorporating a blend of
ultrastructural and antigenic assessments through transmission electron
microscopy, immunofluorescence antigenic mapping, and investigations
utilizing EB-specific monoclonal antibodies(8). In the treatment of this
child, due to a skin disorder, the use of CPAP was not possible.
Additionally, vascular access was not performed, and an umbilical
catheter was placed. Antibiotics were administered due to suspicion of a
skin infection with Staphylococcus aureus, and the treatment was carried
out with ampicillin and amikacin.
Management of Bart syndrome involves a comprehensive approach, including
conservative measures, secondary intention healing, and surgical
interventions when warranted. Traditional care primarily focuses on
localized wound treatment and infection control using systemic
antibiotics(5). The routine administration of systemic antibiotics is
not a standard practice, but they may be considered if there are
concerns about infection. While conservative methods suffice for most
patients, those with substantial or deep wounds may require surgical
interventions such as skin grafting or local flap procedures(5).
Conclusion:
Bart syndrome, an infrequent congenital skin disorder characterized by
its distinct clinical features, emphasizes prompt and conservative
management’s importance in optimizing outcomes. Vigilant patient
monitoring is advised for tracking progress. The first examination
of newborns Can guide us to critical Situations. Some Signs are straight
to the diagnosis, but others are conflicting, and one Should. Search for
another disorder already in Syndromes, so physicians Should be aware of
Many syndromes and associations so as not to miss the exact diagnosis.
Keywords: Pediatric, Bart, Syndrome, Anomaly