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Clinical and Basic Immunodermatology pp 645–652Cite as

Epidermolysis Bullosa Acquisita

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Abstract

In 1895, two cases of a blistering disease with adult onset and features highly reminiscent of hereditary dystrophic epidermolysis bullosa (EB) were reported by Elliott. These clinical features included skin fragility, erosions, blisters, and a healing response characterized by scarring and the formation of milial cysts.

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Author information

Authors and Affiliations

  1. Department of Dermatology, LAC & USC Medical Center, Stanford Hospital, Los Angeles, CA, USA

    Brittney De Clerck MD

  2. Department of Dermatology, USA Norris Cancer Center, Los Angeles, CA, USA

    Mei Chen MD

  3. Department of Dermatology, USA Norris Cancer Center, 1441 East Lake Avenue, Topping Tower #3405, Los Angeles, CA, 90033, USA

    David T. Woodley MD

Authors
  1. Brittney De Clerck MD
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  2. Mei Chen MD
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  3. David T. Woodley MD
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Corresponding author

Correspondence to David T. Woodley MD .

Editor information

Editors and Affiliations

  1. School of Medicine, University of Maryland Baltimore, Baltimore, Maryland, USA

    Anthony A. Gaspari

  2. Department of Dermatology, University of Texas Health Science Center, Houston, Texas, USA

    Stephen K. Tyring

  3. Department of Dermatology and Immunology, University of Pittsburgh, Pittsburgh, Pennsylvania, USA

    Daniel H. Kaplan

Questions

Questions

  1. 1.

    Which of the following tests support the diagnosis of EBA:

    1. A.

      Salt-split skin direct immunofluorescence (DIF) with the IgG deposits going to the epidermal roof

    2. B.

      Immuno-electron microscopy with the IgG immune deposits localized within and below the lamina densa

    3. C.

      Serum indirect immunofluorescence (IIF) showing an antibody that labels the roof of the epidermal- dermal separation

    4. D.

      Anti-endomysial IgA autoantibodies

  2. 2.

    Autoantibodies in EBA patient sera are usually IgG and most commonly bind to which of the following structures in the skin?

    1. A.

      Anchoring plaques

    2. B.

      Integrin a6b4 emanating from the hemidesmosome into the lamina lucida

    3. C.

      Anchoring filaments

    4. D.

      Anchoring fibrils

  3. 3.

    Of the possible underlying systemic diseases associated with EBA, which one is the most common?

    1. A.

      Amyloidosis

    2. B.

      Bullous amyloidosis

    3. C.

      Inflammatory bowel disease

    4. D.

      Lymphoma

  4. 4.

    Regarding EBA treatment, which of the following is true?

    1. A.

      The mechanobullous form of EBA usually responds nicely to high doses of systemic corticosteroids

    2. B.

      Colchicine is a good first line drug in EBA patients with underlying inflammatory bowel disease

    3. C.

      Cyclosporine A can be used to treat EBA at doses similar to those used in psoriasis patients

    4. D.

      Rituximab infusions are a useful therapy for EBA

  5. 5.

    Patients with Brunsting-Perry Pemphigoid usually have:

    1. A.

      Serum autoantibodies to the BP 180 antigen

    2. B.

      Serum autoantibodies to type XVII collagen

    3. C.

      Serum autoantibodies that label the floor of salt-split human skin substrate

    4. D.

      Serum antibodies that only bind to proteins within mucous membranes

Answers

  1. 1.

    B

  2. 2.

    D

  3. 3.

    C

  4. 4.

    D

  5. 5.

    C

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De Clerck, B., Chen, M., Woodley, D.T. (2017). Epidermolysis Bullosa Acquisita. In: Gaspari, A., Tyring, S., Kaplan, D. (eds) Clinical and Basic Immunodermatology. Springer, Cham. https://doi.org/10.1007/978-3-319-29785-9_36

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  • DOI: https://doi.org/10.1007/978-3-319-29785-9_36

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  • Online ISBN: 978-3-319-29785-9

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