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Update on the pathogenesis of bullous pemphigoid: an autoantibody-mediated blistering disease targeting collagen XVII.
J Dermatol Sci. 2014 Mar; 73(3):179-86.JD

Abstract

Bullous pemphigoid (BP) is a common autoimmune blistering skin disorder that tends to affect the elderly. Autoantibodies (autoAbs) from BP patients react with two hemidesmosomal components: transmembrane collagen XVII (BP180 or BPAG2) and plakin family protein BP230 (BPAG1). Of these, collagen XVII (COL17) is thought to be a major autoantigen. The binding of autoAbs to COL17 following the activation of complements and inflammatory pathways eventually leads to the degradation of COL17, and this has been regarded as the main pathogenesis of BP. However, recent investigations have suggested other pathways, including a complement-independent pathway and a pathway involving IgE-autoAbs. BP-autoAbs can directly deplete COL17, leading to fragility of the dermal-epidermal junction. In addition, IgE-autoAbs to COL17 may be involved in the formation of itchy urticarial erythema associated with eosinophilic infiltration. This article summarizes the update on pathogenesis of BP, with a special focus on blister formation by autoAbs to COL17.

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Authors+Show Affiliations

Nishie W
Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan. Electronic address: nishie@med.hokudai.ac.jp.

MeSH

Amino Acid SequenceAnimalsAutoantibodiesAutoantigensComplement System ProteinsHumansImmunoglobulin EInflammationMolecular Sequence DataNon-Fibrillar CollagensPemphigoid, Bullous

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

24434029

Citation

Nishie, Wataru. "Update On the Pathogenesis of Bullous Pemphigoid: an Autoantibody-mediated Blistering Disease Targeting Collagen XVII." Journal of Dermatological Science, vol. 73, no. 3, 2014, pp. 179-86.
Nishie W. Update on the pathogenesis of bullous pemphigoid: an autoantibody-mediated blistering disease targeting collagen XVII. J Dermatol Sci. 2014;73(3):179-86.
Nishie, W. (2014). Update on the pathogenesis of bullous pemphigoid: an autoantibody-mediated blistering disease targeting collagen XVII. Journal of Dermatological Science, 73(3), 179-86. https://doi.org/10.1016/j.jdermsci.2013.12.001
Nishie W. Update On the Pathogenesis of Bullous Pemphigoid: an Autoantibody-mediated Blistering Disease Targeting Collagen XVII. J Dermatol Sci. 2014;73(3):179-86. PubMed PMID: 24434029.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Update on the pathogenesis of bullous pemphigoid: an autoantibody-mediated blistering disease targeting collagen XVII. A1 - Nishie,Wataru, Y1 - 2013/12/27/ PY - 2013/11/15/received PY - 2013/12/04/accepted PY - 2014/1/18/entrez PY - 2014/1/18/pubmed PY - 2014/10/18/medline KW - Autoantibody KW - Complement activation KW - IgE KW - Model mice KW - Protease SP - 179 EP - 86 JF - Journal of dermatological science JO - J Dermatol Sci VL - 73 IS - 3 N2 - Bullous pemphigoid (BP) is a common autoimmune blistering skin disorder that tends to affect the elderly. Autoantibodies (autoAbs) from BP patients react with two hemidesmosomal components: transmembrane collagen XVII (BP180 or BPAG2) and plakin family protein BP230 (BPAG1). Of these, collagen XVII (COL17) is thought to be a major autoantigen. The binding of autoAbs to COL17 following the activation of complements and inflammatory pathways eventually leads to the degradation of COL17, and this has been regarded as the main pathogenesis of BP. However, recent investigations have suggested other pathways, including a complement-independent pathway and a pathway involving IgE-autoAbs. BP-autoAbs can directly deplete COL17, leading to fragility of the dermal-epidermal junction. In addition, IgE-autoAbs to COL17 may be involved in the formation of itchy urticarial erythema associated with eosinophilic infiltration. This article summarizes the update on pathogenesis of BP, with a special focus on blister formation by autoAbs to COL17. SN - 1873-569X UR - https://www.unboundmedicine.com/medline/citation/24434029/Update_on_the_pathogenesis_of_bullous_pemphigoid:_an_autoantibody_mediated_blistering_disease_targeting_collagen_XVII_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0923-1811(13)00386-1 DB - PRIME DP - Unbound Medicine ER -