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Clinical Manifestations and Complications

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Life with Epidermolysis Bullosa (EB)

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Abstract

The hallmark feature of inherited EB is mechanical fragility. Invariably this is associated with the development of erosions. In most forms of EB these erosions are preceded by tense blisters, most of which are filled with clear, colorless exudate. Occasionally these blisters may be hemorrhagic, most notably in the rare Ogna subtype of EBS. In the rather newly described rare EB subtypes characterized by cleavage within the uppermost portion of the epidermis (“suprabasal EBS subtypes”), which include EBS superficialis [3], lethal acantholytic EBS [6], and plakophilin-1 deficiency [7], intact blisters are usually not clinically evident, making diagnosis sometimes confusing on the basis of skin findings alone.

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Authors and Affiliations

  1. Department of Medicine (Dermatology) & Pediatrics, Vanderbilt University School of Medicine, USA

    Prof. Jo-David Fine (Head)

  2. National (USA) Epidermolysis Bullosa Registry, Nashville, TN, USA

    Prof. Jo-David Fine (Head)

  3. Universitätsklinik für Dermatologie, Paracelsus Medizinische Privatuniversität Salzburg, Salzburg, Austria

    Dr. Christoph M. Lanschuetzer, Dr. Martin Laimer & Dr. Elke Nischler

  4. Universitätsklinik für Augenheilkunde und Optometrie, Paracelsus Medizinische Privatuniversität Salzburg, Salzburg, Austria

    Univ.-Doz. Dr. Josef Stoiber

Authors
  1. Prof. Jo-David Fine
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  2. Dr. Christoph M. Lanschuetzer
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  3. Univ.-Doz. Dr. Josef Stoiber
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  4. Dr. Martin Laimer
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  5. Dr. Elke Nischler
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Editor information

Editors and Affiliations

  1. Division of Dermatology, Department of Medicine, Vanderbilt University School of Medicine, and National EB Registry, Nashville, Tennessee, USA

    Jo-David Fine

  2. Universitätsklinik für Dermatologie, Paracelsus Medizinische Privatuniversität Salzburg, Salzburg, Österreich

    Helmut Hintner

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© 2009 Springer-Verlag/Wien

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Fine, JD., Lanschuetzer, C.M., Stoiber, J., Laimer, M., Nischler, E. (2009). Clinical Manifestations and Complications. In: Fine, JD., Hintner, H. (eds) Life with Epidermolysis Bullosa (EB). Springer, Vienna. https://doi.org/10.1007/978-3-211-79271-1_2

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  • DOI: https://doi.org/10.1007/978-3-211-79271-1_2

  • Publisher Name: Springer, Vienna

  • Print ISBN: 978-3-211-79270-4

  • Online ISBN: 978-3-211-79271-1

  • eBook Packages: MedicineMedicine (R0)

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