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[Anti-p200 pemphigoid--a new bullous autoimmune dermatosis].
J Dtsch Dermatol Ges. 2004 Jan; 2(1):7-14.JD

Abstract

Anti-p200 pemphigoid is an autoimmune skin disease characterized by tense blisters, subepidermal split formation, and mainly neutrophilic inflammatory infiltration of the dermal-epidermal junction (DEJ). Direct immunofluorescence microscopy of perilesional skin biopsies demonstrates linear deposits of IgG and C3 along the DEJ, while by indirect immunofluorescence microscopy on NaCI-split human skin, patients' IgG labels the dermal side. The antigenic target of the autoantibodies is a 200 kD protein (p200) of the lower lamina lucida that can be detected in human dermal extracts by immunoblotting. While p200 is thought to be important for cell-matrix adhesion, its exact identity is unknown. To date, the p200 autoantigen has been demonstrated to be distinct from bullous pemphigoid antigens 180 und 230, laminin 1, 5, and 6, alpha6beta4 integrin, and type VII collagen. Biochemical characterization of the p200 molecule revealed a noncollagenous N-glycosylated acidic protein with an isoelectric point of approximately 5.5. We provide an overview on pathogenesis, clinical features, diagnosis, and treatment of this unique autoimmune dermatosis.

Authors+Show Affiliations

Universitäts-Hautklinik Würzburg, Germany.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

English Abstract
Journal Article
Review

Language

ger

PubMed ID

16281877

Citation

Shimanovich, Iakov, et al. "[Anti-p200 Pemphigoid--a New Bullous Autoimmune Dermatosis]." Journal Der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG, vol. 2, no. 1, 2004, pp. 7-14.
Shimanovich I, Rose C, Hirako Y, et al. [Anti-p200 pemphigoid--a new bullous autoimmune dermatosis]. J Dtsch Dermatol Ges. 2004;2(1):7-14.
Shimanovich, I., Rose, C., Hirako, Y., Butt-Dörje, E., & Zillikens, D. (2004). [Anti-p200 pemphigoid--a new bullous autoimmune dermatosis]. Journal Der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG, 2(1), 7-14.
Shimanovich I, et al. [Anti-p200 Pemphigoid--a New Bullous Autoimmune Dermatosis]. J Dtsch Dermatol Ges. 2004;2(1):7-14. PubMed PMID: 16281877.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Anti-p200 pemphigoid--a new bullous autoimmune dermatosis]. AU - Shimanovich,Iakov, AU - Rose,Christian, AU - Hirako,Yoshiaki, AU - Butt-Dörje,Elke, AU - Zillikens,Detlef, PY - 2005/11/12/pubmed PY - 2005/12/21/medline PY - 2005/11/12/entrez SP - 7 EP - 14 JF - Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG JO - J Dtsch Dermatol Ges VL - 2 IS - 1 N2 - Anti-p200 pemphigoid is an autoimmune skin disease characterized by tense blisters, subepidermal split formation, and mainly neutrophilic inflammatory infiltration of the dermal-epidermal junction (DEJ). Direct immunofluorescence microscopy of perilesional skin biopsies demonstrates linear deposits of IgG and C3 along the DEJ, while by indirect immunofluorescence microscopy on NaCI-split human skin, patients' IgG labels the dermal side. The antigenic target of the autoantibodies is a 200 kD protein (p200) of the lower lamina lucida that can be detected in human dermal extracts by immunoblotting. While p200 is thought to be important for cell-matrix adhesion, its exact identity is unknown. To date, the p200 autoantigen has been demonstrated to be distinct from bullous pemphigoid antigens 180 und 230, laminin 1, 5, and 6, alpha6beta4 integrin, and type VII collagen. Biochemical characterization of the p200 molecule revealed a noncollagenous N-glycosylated acidic protein with an isoelectric point of approximately 5.5. We provide an overview on pathogenesis, clinical features, diagnosis, and treatment of this unique autoimmune dermatosis. SN - 1610-0379 UR - https://www.unboundmedicine.com/medline/citation/16281877/[Anti_p200_pemphigoid__a_new_bullous_autoimmune_dermatosis]_ L2 - https://doi.org/10.1046/j.1439-0353.2004.03047.x DB - PRIME DP - Unbound Medicine ER -