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[Anti-p200 pemphigoid].
Hautarzt. 2019 Apr; 70(4):271-276.H

Abstract

Anti-p200 pemphigoid is a rare autoimmune blistering disease. It belongs to the group of pemphigoid diseases and was first described in 1996. The diagnostic gold standard is the combination of (1) linear deposits of immunoreactants at the dermal epidermal junction by direct immunofluorescence microscopy of a perilesional skin biopsy, (2) detection of circulating autoantibodies binding to the dermal side (blister floor) of human salt split skin by indirect immunofluorescence microscopy, and reactivity with a 200 kDa protein (p200) in extract of human dermis by immunoblotting. In 2009, laminin γ1 was described as an additional target antigen in 90% of anti-p200 pemphigoid patients. Since ex vivo and in vivo studies have shown no direct pathogenic relevance for laminin γ1 antibodies and the preadsorption of patient sera against laminin γ1 does not reduce their reactivity with p200, the molecular identity of p200 still remains to be elucidated. The clinical phenotype of the disease is heterogeneous; in most cases, however, it resembles bullous pemphigoid. Anti-p200 patients are younger and skin lesions more often appear on palms of the hands and soles of the feet than in bullous pemphigoid. Therapy consists of topical and systemic corticosteroids. In addition, the use of daspone and immunosuppressants has been reported.

Authors+Show Affiliations

Klinik für Dermatologie, Allergologie und Venerologie, Universität zu Lübeck, Ratzeburger Allee 160, 23538, Lübeck, Deutschland. maikemarleen.holtsche@uksh.de.Klinik für Dermatologie, Allergologie und Venerologie, Universität zu Lübeck, Ratzeburger Allee 160, 23538, Lübeck, Deutschland.Klinik für Dermatologie, Allergologie und Venerologie, Universität zu Lübeck, Ratzeburger Allee 160, 23538, Lübeck, Deutschland.

Pub Type(s)

Journal Article
Review

Language

ger

PubMed ID

30868255

Citation

Holtsche, M M., et al. "[Anti-p200 Pemphigoid]." Der Hautarzt; Zeitschrift Fur Dermatologie, Venerologie, Und Verwandte Gebiete, vol. 70, no. 4, 2019, pp. 271-276.
Holtsche MM, Goletz S, Zillikens D. [Anti-p200 pemphigoid]. Hautarzt. 2019;70(4):271-276.
Holtsche, M. M., Goletz, S., & Zillikens, D. (2019). [Anti-p200 pemphigoid]. Der Hautarzt; Zeitschrift Fur Dermatologie, Venerologie, Und Verwandte Gebiete, 70(4), 271-276. https://doi.org/10.1007/s00105-019-4376-x
Holtsche MM, Goletz S, Zillikens D. [Anti-p200 Pemphigoid]. Hautarzt. 2019;70(4):271-276. PubMed PMID: 30868255.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Anti-p200 pemphigoid]. AU - Holtsche,M M, AU - Goletz,S, AU - Zillikens,D, PY - 2019/3/15/pubmed PY - 2019/9/10/medline PY - 2019/3/15/entrez KW - Autoantibodies KW - Basement membrane zone KW - Blister KW - Immunofluorescence KW - Immunosuppression SP - 271 EP - 276 JF - Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete JO - Hautarzt VL - 70 IS - 4 N2 - Anti-p200 pemphigoid is a rare autoimmune blistering disease. It belongs to the group of pemphigoid diseases and was first described in 1996. The diagnostic gold standard is the combination of (1) linear deposits of immunoreactants at the dermal epidermal junction by direct immunofluorescence microscopy of a perilesional skin biopsy, (2) detection of circulating autoantibodies binding to the dermal side (blister floor) of human salt split skin by indirect immunofluorescence microscopy, and reactivity with a 200 kDa protein (p200) in extract of human dermis by immunoblotting. In 2009, laminin γ1 was described as an additional target antigen in 90% of anti-p200 pemphigoid patients. Since ex vivo and in vivo studies have shown no direct pathogenic relevance for laminin γ1 antibodies and the preadsorption of patient sera against laminin γ1 does not reduce their reactivity with p200, the molecular identity of p200 still remains to be elucidated. The clinical phenotype of the disease is heterogeneous; in most cases, however, it resembles bullous pemphigoid. Anti-p200 patients are younger and skin lesions more often appear on palms of the hands and soles of the feet than in bullous pemphigoid. Therapy consists of topical and systemic corticosteroids. In addition, the use of daspone and immunosuppressants has been reported. SN - 1432-1173 UR - https://www.unboundmedicine.com/medline/citation/30868255/[Anti_p200_pemphigoid]_ L2 - https://dx.doi.org/10.1007/s00105-019-4376-x DB - PRIME DP - Unbound Medicine ER -