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Research paper| Volume 14, ISSUE 1, P68-75, January 1997

The role of immunohistochemistry in the diagnosis of the non-lethal forms of junctional epidermolysis bullosa

  • John A. McGrath
    Affiliations
    Department of Cell Pathology, St John's Institute of Dermatology, St Thomas's Hospital, Lambeth Palace Road, London SEl7EH, UK
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  • Robin A.J. Eady
    Correspondence
    Corresponding author. Tel.: +44 171 9289292, ext. 3318; fax: +44 171 9228175.
    Affiliations
    Department of Cell Pathology, St John's Institute of Dermatology, St Thomas's Hospital, Lambeth Palace Road, London SEl7EH, UK
    Search for articles by this author
DOI:https://doi.org/10.1016/S0923-1811(96)00554-3
The role of immunohistochemistry in the diagnosis of the non-lethal forms of junctional epidermolysis bullosa
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      Abstract

      Skin biopsies from four patients with similar clinical phenotypes of non-lethal junctional epidermolysis bullosa (JEB) were investigated by electron microscopy and immunohistochemistry. Electron microscopy revealed cleavage within the lamina lucida in each case, but in addition, there was considerable variability in the number and ultrastructure of hemidesmosome-anchoring filament complexes. Immunohistochemical staining of the dermal-epidermal junction, using monoclonal antibodies to laminin 5 and the 180-kDa bullous pemphigoid antigen, showed reduced laminin 5 staining with normal 180-kDa bullous pemphigoid antigen labelling in two cases, and reduced or absent 180-kDa bullous pemphigoid antigen staining with normal laminin 5 labelling in the other two cases. Whereas electron microscopy is invaluable for the diagnosis of JEB as a group of diseases, immunohistochemistry is able to provide insight into the molecular pathology of non-lethal JEB and reveal abnormalities in candidate genes and proteins underlying this group of genetic blistering disorders.

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      Article info

      Publication history

      Accepted: April 22, 1996
      Received in revised form: March 26, 1996
      Received: January 30, 1996

      Identification

      DOI: https://doi.org/10.1016/S0923-1811(96)00554-3

      Copyright

      © 1997 Published by Elsevier Inc.

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