Dystrophic Epidermolysis Bullosa Associated with Amniotic Band Syndrome

Authors

  • Haley Snadecki B.A.,

    1. Division of Dermatology, Department of Medicine, University of Massachusetts Medical School, Worcester, Massachusetts
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  • Vincent Criscione M.D.,

    1. Division of Dermatology, Department of Medicine, University of Massachusetts Medical School, Worcester, Massachusetts
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  • Alisha Jaquith M.D.,

    1. Department of Psychiatry, University of Massachusetts Medical School, Worcester, Massachusetts
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  • Beverly Hay M.D.,

    1. Department of Pediatrics, University of Massachusetts Medical School, Worcester, Massachusetts
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  • April Deng M.D.,

    1. Department of Pathology, University of Massachusetts Medical School, Worcester, Massachusetts
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  • Karen Wiss M.D.

    Corresponding author
    1. Division of Dermatology, Department of Medicine, University of Massachusetts Medical School, Worcester, Massachusetts
    2. Department of Pediatrics, University of Massachusetts Medical School, Worcester, Massachusetts
    • Address correspondence to Karen Wiss, M.D., Director, Pediatric Dermatology, Departments of Medicine and Pediatrics, Division of Dermatology, University of Massachusetts Medical School, 281 Lincoln Street, Worcester, MA 01605, or

      e-mail: Karen.wiss@umassmemorial.org.

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Abstract

Amniotic band syndrome (ABS) is a term used to describe congenital anomalies that result from the entrapment of a fetus in fibrous bands. We describe two male infants born with features of dystrophic epidermolysis bullosa (DEB) and ABS. These cases add to the few previous reports of simultaneous DEB and ABS. Abnormal type VII collagen in anchoring structures of the amniotic epithelium is a proposed mechanism for loose amniotic bands that entangle the fetus, with an abnormality in the gene that encodes for type VII collagen.

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