Type VI Aplasia Cutis Congenita: Bart’s Syndrome

Abstract

Bart's syndrome is characterized by aplasia cutis congenita and epidermolysis bullosa. We present the case of a newborn male who developed blisters on the mucous membranes and the skin following congenital localized absence of skin. Bart's syndrome (BS) is diagnosed clinically based on the disorder's unique signs and symptoms but histologic evaluation of the skin can help to confirm the final diagnosis. The patient was managed conservatively with topical antibacterial ointment and wet gauze dressing. Periodic follow-up examinations showed complete healing. We emphasized that it is important to use relatively simple methods for optimal healing without the need for complex surgical interventions.

A Case Report of Aplasia Cutis Congenita Type VI: Bart Syndrome

“…Th e inheritance pattern of Bart's syndrome seems to be autosomal dominant. Nevertheless, several sporadic cases have been reported 6 . Bart syndrome has no known underlying cause.…”

“…Bart's syndrome is a rare congenital skin disorder characterized by a unique clinical presentation. Looking for other associated anomalies is necessary 6 . In gen eral, the syndrome has a good prognosis, but it should be managed as early as possible to reach the best outcome.…”

“…Th e prognosis in Bart's syndrome depends on a large number of factors, like the severity of aplasia cutis, epidermolysis bullosa subtype, associated anomalies, and effi cacy of treatment. Generally, the prognosis is good 6 . Th e patients have a normal life expectancy and in almost all cases they have a normal life.…”

A cost-effective treatment model in dystrophic epidermolysis bullosa with congenital absence of skin

“…In the literature, the cases with Bart's syndrome with an extensive skin loss were reported to be treated with Bactigras® dressing and such materials due to skin loss once or twice a day (Badger & O'Haver, ; Kulalı et al, ). Although the cost of this wound care set varies considerably from country to country, it has a significant burden on the hospitalization costs, particularly in cases where it is needed for long‐term use, such as in our case.…”

A case of Bart syndrome

“…The prognosis of BS is considered to be good and they have normal life expectancy. However, it is important to protect these children from hypothermia, infections and excessive friction 6 .…”