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Epidermolysis Bullosa Acquisita: Dermatological Features

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Abstract

Clinical signs and features include:

Incidence: 0.2–0.5/million persons/year [1]; increased in persons of sub-Saharan African descent (African Americans); mean onset is age 50

Classic: skin fragility, tense blisters on trauma prone extensor surfaces, noninflamed skin with milia cysts and atrophic scars when healed, scarring alopecia, nail dystrophy, rare oral mucosal involvement

Inflammatory: widespread tense blisters surrounded by urticarial plaques associated with pruritus

Cicatricial pemphigoid like form: mucosal involvement with lesions in the mouth, conjunctiva, nose, larynx, genitalia, anus, and esophagus (strictures in proximal third leading to dysphagia)

Brunsting/Perry pemphigoid-like form: vesicles and blisters only on the head, neck, and upper aspect of the trunk, with no mucosal involvement

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References

  1. Caux F. Diagnosis and clinical features of epidermolysis bullosa acquisita. Dermatol Clin. 2011;29:485–91.

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Author information

Authors and Affiliations

  1. Yale Department of Internal Medicine, Yale New Haven Hospital, 20 York Street, New Haven, CT, 06510, USA

    Liam Zakko

  2. Department of Dermatology, University of Connecticut Health Center, 21 South Road, Farmington, CT, 06030, USA

    Justin Finch, Marti J. Rothe & Jane M. Grant-Kels

Authors
  1. Liam Zakko
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  2. Justin Finch
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  3. Marti J. Rothe
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  4. Jane M. Grant-Kels
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Corresponding author

Correspondence to Liam Zakko .

Editor information

Editors and Affiliations

  1. Department of Medicine, Gastroenterology Division,, University of Connecticut Health Center, 263 Farmington Ave., Farmington, 06030, Connecticut, USA

    George Y. Wu

  2. , Department of Medicine, University of Connecticut, 56 Kingswood Road, West Hartford, 06119, Connecticut, USA

    Nathan Selsky

  3. , Univ. of CT Health Ctr, Dermatol. Dept., University of Connecticut, 21 South Road, Farmington, 06032, Connecticut, USA

    Jane M. Grant-Kels

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Zakko, L., Finch, J., Rothe, M.J., Grant-Kels, J.M. (2013). Epidermolysis Bullosa Acquisita: Dermatological Features. In: Wu, G., Selsky, N., Grant-Kels, J. (eds) Atlas of Dermatological Manifestations of Gastrointestinal Disease. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-6191-3_10

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  • DOI: https://doi.org/10.1007/978-1-4614-6191-3_10

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  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4614-6190-6

  • Online ISBN: 978-1-4614-6191-3

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