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A Case of Bart's Syndrome - Epidermolysis Bullosa and Congenital Localized Absence of Skin -

Journal of the Korean Pediatric Society 1994;37(1):104-108.
Published online January 15, 1994.
A Case of Bart's Syndrome - Epidermolysis Bullosa and Congenital Localized Absence of Skin -
Hong Shin Jeon1, Young Jin Hong1, Don Hee Ahn1, Hee Jun Yoo2, Je Geun Chi3
1Department of Pediatrics, National Medical Center, Seoul, Korea
2Department of Dermatology, National Medical Center, Seoul, Korea
3Department of Pathology, Seoul National University College of Medicine, Seoul, Korea
Bart 증후군 1례
전홍신1, 홍영진1, 안돈희1, 유희준2, 지제근3
1국립의료원 소아과
2국립의료원 피부과
3서울대학교 의과대학 병리학교실
Abstract
A female newborn had the following characteristics: a congenital localized absence of skin over the lower extremities; blistering of the skin or mucous membrane, incited by trauma, which heals without scarring; and congenital absence or deformity of the nails. In respect to the characteristic manifestationhj, clinical course and electron microscopic features, our patient seems to fit well into Bart's syndrome. The inheritance pattern appears to be autosomal dominant but, as in this report, isolated cases have been recognized. As the review of the literatures, congenital localized absence of skin has been observed in various subsets of inherited E.B. But, we believe that the term Bart's syndrome should be used to identify patients with good prognosis as the Bart's initial description. To the best of our knowedge, this is the first reported case of Bart's syndrome in the korean literature.
Key Words: Bart's syndrome, Epidermolysis bullosa, congenital localized absence of skin


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