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Is Bart's Syndrome a Specific Syndrome?-Reply

Is Bart's Syndrome a Specific Syndrome?-Reply This article is only available in the PDF format. Download the PDF to view the article, as well as its associated figures and tables. Abstract 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 http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Is Bart's Syndrome a Specific Syndrome?-Reply

Kanzler, Matthew H.; Smoller, Bruce
Archives of Dermatology , Volume 129 (7) – Jul 1, 1993
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Is Bart's Syndrome a Specific Syndrome?-Reply

Abstract

This article is only available in the PDF format. Download the PDF to view the article, as well as its associated figures and tables. Abstract 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...
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Publisher
American Medical Association
Copyright
Copyright © 1993 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.1993.01680280098022
Publisher site
See Article on Publisher Site

Abstract

This article is only available in the PDF format. Download the PDF to view the article, as well as its associated figures and tables. Abstract 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

Journal

Archives of Dermatology – American Medical Association

Published: Jul 1, 1993

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