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Anti-p200 pemphigoid.
J Am Acad Dermatol. 2014 Jul; 71(1):185-91.JA

Abstract

Anti-p200 pemphigoid is a rare subepidermal blistering skin disease. Patients' autoantibodies label the dermal side of 1 mol/L NaCl-split human skin by indirect immunofluorescence microscopy and recognize a 200-kd protein by immunoblotting of human dermal extract. Clinically, anti-p200 pemphigoid is characterized by tense blisters and vesicles, erosions, and urticarial plaques, closely resembling bullous pemphigoid and the inflammatory variant of epidermolysis bullosa acquisita. Recently, 90% of anti-p200 pemphigoid sera were shown to recognize laminin γ1. The C-terminus of laminin γ1 was identified as an immunodominant region and in its recombinant form was used by immunoblotting and enzyme-linked immunosorbent assay for the serologic diagnosis of this disease. Subsequent ex vivo and in vivo studies were, however, unable to show pathogenic activity of antilaminin γ1 antibodies. Both patients' sera and sera depleted from antilaminin γ1 antibodies induced subepidermal splitting in an ex vivo model of autoantibody-mediated leukocyte-dependent neutrophil activation. Antilaminin γ1 antibodies appear to be useful biomarkers that will further facilitate the diagnosis of anti-p200 pemphigoid. The true identity of the pathogenetically relevant autoantigen of this disease, which may either be a yet unknown isoform of laminin γ1 or even another 200-kd protein of the dermoepidermal junction, still needs to be elucidated.

Authors+Show Affiliations

Department of Dermatology, University of Luebeck, Luebeck, Germany.Department of Dermatology, Kurume University School of Medicine, Kurume, Japan.Department of Dermatology, University of Luebeck, Luebeck, Germany.Department of Dermatology, University of Luebeck, Luebeck, Germany. Electronic address: enno.schmidt@uksh.de.

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't
Review

Language

eng

PubMed ID

24767733

Citation

Goletz, Stephanie, et al. "Anti-p200 Pemphigoid." Journal of the American Academy of Dermatology, vol. 71, no. 1, 2014, pp. 185-91.
Goletz S, Hashimoto T, Zillikens D, et al. Anti-p200 pemphigoid. J Am Acad Dermatol. 2014;71(1):185-91.
Goletz, S., Hashimoto, T., Zillikens, D., & Schmidt, E. (2014). Anti-p200 pemphigoid. Journal of the American Academy of Dermatology, 71(1), 185-91. https://doi.org/10.1016/j.jaad.2014.02.036
Goletz S, et al. Anti-p200 Pemphigoid. J Am Acad Dermatol. 2014;71(1):185-91. PubMed PMID: 24767733.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Anti-p200 pemphigoid. AU - Goletz,Stephanie, AU - Hashimoto,Takashi, AU - Zillikens,Detlef, AU - Schmidt,Enno, Y1 - 2014/04/24/ PY - 2013/12/13/received PY - 2014/02/17/revised PY - 2014/02/19/accepted PY - 2014/4/29/entrez PY - 2014/4/29/pubmed PY - 2014/9/10/medline KW - anti-p200 pemphigoid KW - autoantibody KW - autoantigen KW - laminin γ1 KW - p200 antigen KW - subepidermal blistering skin disease SP - 185 EP - 91 JF - Journal of the American Academy of Dermatology JO - J Am Acad Dermatol VL - 71 IS - 1 N2 - Anti-p200 pemphigoid is a rare subepidermal blistering skin disease. Patients' autoantibodies label the dermal side of 1 mol/L NaCl-split human skin by indirect immunofluorescence microscopy and recognize a 200-kd protein by immunoblotting of human dermal extract. Clinically, anti-p200 pemphigoid is characterized by tense blisters and vesicles, erosions, and urticarial plaques, closely resembling bullous pemphigoid and the inflammatory variant of epidermolysis bullosa acquisita. Recently, 90% of anti-p200 pemphigoid sera were shown to recognize laminin γ1. The C-terminus of laminin γ1 was identified as an immunodominant region and in its recombinant form was used by immunoblotting and enzyme-linked immunosorbent assay for the serologic diagnosis of this disease. Subsequent ex vivo and in vivo studies were, however, unable to show pathogenic activity of antilaminin γ1 antibodies. Both patients' sera and sera depleted from antilaminin γ1 antibodies induced subepidermal splitting in an ex vivo model of autoantibody-mediated leukocyte-dependent neutrophil activation. Antilaminin γ1 antibodies appear to be useful biomarkers that will further facilitate the diagnosis of anti-p200 pemphigoid. The true identity of the pathogenetically relevant autoantigen of this disease, which may either be a yet unknown isoform of laminin γ1 or even another 200-kd protein of the dermoepidermal junction, still needs to be elucidated. SN - 1097-6787 UR - https://www.unboundmedicine.com/medline/citation/24767733/Anti_p200_pemphigoid_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0190-9622(14)01153-0 DB - PRIME DP - Unbound Medicine ER -