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 manifestation, 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.
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