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August 1992

Congenital Localized Absence of the Skin as a Manifestation of Epidermolysis Bullosa

Author Affiliations

From the Division of Dermatology, Santa Clara Valley Medical Center, San Jose, Calif (Dr Kanzler); Departments of Dermatology (Drs Kanzler and Woodley) and Pathology (Dr Smoller), Stanford (Calif) University Medical Center.

Arch Dermatol. 1992;128(8):1087-1090. doi:10.1001/archderm.1992.01680180081010

• Background.—  Congenital localized absence of the skin has been observed in various subsets of inherited epidermolysis bullosa. Through electron microscopy and immunomapping, we attempt to clarify the relationship of congenital localized absence of the skin lesions to epidermolysis bullosa.

Observations.—  The case of a child with epidermolysis bullosa simplex and congenital localized absence of the skin is presented. Electron microscopy and immunomapping of the areas of congenital localized absence of the skin and sites of skin fragility suggest that these lesions are pathogenically identical.

Conclusion.—  After reviewing the literature, we believe that the term Bart's syndrome should be used to identify patients with any type of epidermolysis bullosa who present with localized congenital absence of the skin on the extremities.(Arch Dermatol. 1992;128:1087-1090)

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