Bart Syndrome: a rare entity

Authors

  • Chitreddy Uma Reddy
  • Komar Suresh Reddy
  • Jaddu Jyothirmai Reddy

DOI:

https://doi.org/10.7213/aor.v7i1.23146

Keywords:

Bart Syndrome, Epidermolysis Bullosa Dystrophica, Transient bullous dermolysis of the newborn, Oral manifestations

Abstract


Introduction: Bart Syndrome is a rare inherited skin blistering disorder. It is also known as congenital transient mechano-bullous dermatosis and is one of the lesser known presentations of epidermolysis bullosa (EB). Case report: The objective of this report is to present a case of Bart Syndrome in a 3 day old newborn female baby. The skin lesions showed denuded areas with bullae rupturing easily to reveal painful eroded areas. Eroded lesions were distributed over the hands, feet, chest and on the face over the cheeks bilaterally. The lips were erythematous, eroded with tissue tags. Eroded, crustated lesions were seen on the labial mucosa and anterior palate. Histopathological examination revealed split localized to the epidermis. The epidermal layer above the spilt appeared to be normal. The basement membrane was intact, along with normal underlying connective tissue. Discussion: Management consisted of decompression of blisters followed by topical antibiotics. Oral corticosteroids were given for control of blistering, since they reduce collagenase activity. Avoidance of trauma is essential aspect of management: baby was nursed with care to prevent occurrence of new lesions. Therapy and counseling sessions were scheduled for the parents.

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Published

2011-11-28

How to Cite

Reddy, C. U., Reddy, K. S., & Reddy, J. J. (2011). Bart Syndrome: a rare entity. Archives of Oral Research, 7(1). https://doi.org/10.7213/aor.v7i1.23146

Issue

Section

Case Reports