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July 1993

Is Bart's Syndrome a Specific Syndrome?-Reply

Author Affiliations

Division of Dermatology Santa Clara Valley Medical Center San Jose, CA 95128; Department of Pathology Stanford University Medical Center Stanford, CA 94305

Arch Dermatol. 1993;129(7):908. doi:10.1001/archderm.1993.01680280098022

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In Reply.—  We did not "mistakenly" include lethal cases in our list of patients with Bart's syndrome. The inclusion was intentional. Drs Amichai and Metzker agreed that Bart's syndrome has been reported with different modes of inheritance, different clinical findings, and varied electron microscopic and immunohistologic results. Since basically all types of epidermolysis bullosa (EB) have been reported to present with congenital localized absence of the skin (CLAS), there obviously will be some lethal cases of EB reported. Their assumption that Bart's syndrome follows a benign course with eventual arrest of the lesions is false.Although the areas of CLAS do resolve in these patients, the manifestations of the associated EB frequently continue throughout life. If the patients present with a "benign" form of EB, then they will follow a benign course. However, if the patient's manifestations are those of one of the lethal forms of EB, the course is far from benign.We disagree with Drs Amichai and Metzker's contention

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