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Generalized Severe Junctional Epidermolysis Bullosa

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Blistering Diseases

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Abstract

The generalized severe subtype of junctional EB (formerly junctional EB Herlitz type) is the usually most severe lethal variant of the EB group of mechanobullous genodermatoses. Extreme blistering of the skin and mucous membranes and impaired wound healing lead to chronic erosions and formation of exuberant granulation tissue which, in addition to pigmentary changes, nail anomalies, and dental hypoplasia, are clinical hallmarks of the disease. Complications arising from numerous extracutaneous manifestations involving, for instance, the eye and gastrointestinal, laryngeal, and genitourinary tract also account for the devastatingly high morbidity and mortality in early childhood.

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Authors and Affiliations

  1. Department of Dermatology, General Hospital Salzburg, Paracelsus Medical University, Salzburg, Austria

    Martin Laimer MD, Johann W. Bauer MD, MBA & Helmut Hintner MD

Authors
  1. Martin Laimer MD
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  2. Johann W. Bauer MD, MBA
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  3. Helmut Hintner MD
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Corresponding author

Correspondence to Martin Laimer MD .

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Editors and Affiliations

  1. Department of Dermatology, St George Hospital, University of New South Wales, Sydney, New South Wales, Australia

    Dédée F. Murrell

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Laimer, M., Bauer, J.W., Hintner, H. (2015). Generalized Severe Junctional Epidermolysis Bullosa. In: Murrell, D. (eds) Blistering Diseases. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-45698-9_36

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  • DOI: https://doi.org/10.1007/978-3-662-45698-9_36

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  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-662-45697-2

  • Online ISBN: 978-3-662-45698-9

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