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IgA anti-p200 pemphigoid.
Arch Dermatol. 2011 Nov; 147(11):1306-10.AD

Abstract

BACKGROUND

Anti-p200 pemphigoid is a rare autoimmune subepidermal blistering disorder. Clinically, it may resemble bullous pemphigoid, linear IgA bullous dermatosis, or dermatitis herpetiformis. Immunologically, anti-p200 pemphigoid is characterized by the development of IgG antibodies directed against a basement membrane zone protein with a molecular weight of 200 kDa.

OBSERVATIONS

We report the first case, to our knowledge, of anti-p200 pemphigoid associated with IgA antibodies and having clinical features resembling pemphigus herpetiformis or dermatitis herpetiformis localized on traumatized areas. Histopathological examination of lesional skin showed dermal-epidermal separation and microabscesses composed of neutrophils in the dermal papillae. Direct immunofluorescence disclosed the presence exclusively of linear in vivo-bound IgA along the basement membrane zone. With the use of laser scanning confocal microscopy, in vivo-bound IgA was localized above collagen type IV and colocalized with laminin 332. Indirect immunofluorescence showed circulating IgA antibodies against basement membrane zone at a titer of 1:160 that reacted with the floor of an artificial blister of salt-split skin. Western immunoblot analysis using dermal extract confirmed the reactivity of circulating IgA antibodies with the 200-kDa antigen corresponding to laminin γ1; however, immunoblotting using recombinant protein of 107 amino acid C-terminus of laminin γ1 was negative for circulating IgA antibodies. Immunoelectron microscopy disclosed the reactivity of circulating IgA autoantibodies within the lower lamina lucida.

CONCLUSION

To the best of our knowledge, this is the first case fulfilling the immunopathological criteria for anti-p200 pemphigoid associated with IgA antibodies and having unusual clinical features.

Authors+Show Affiliations

Department of Dermatology, Medical University of Warsaw, Koszykowa 82a St, 02-008 Warszawa, Poland. kwozniak@wum.edu.plNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

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

Language

eng

PubMed ID

22106117

Citation

Wozniak, Katarzyna, et al. "IgA Anti-p200 Pemphigoid." Archives of Dermatology, vol. 147, no. 11, 2011, pp. 1306-10.
Wozniak K, Hashimoto T, Fukuda S, et al. IgA anti-p200 pemphigoid. Arch Dermatol. 2011;147(11):1306-10.
Wozniak, K., Hashimoto, T., Fukuda, S., Ohyama, B., Ishii, N., Koga, H., Dainichi, T., & Kowalewski, C. (2011). IgA anti-p200 pemphigoid. Archives of Dermatology, 147(11), 1306-10. https://doi.org/10.1001/archdermatol.2011.303
Wozniak K, et al. IgA Anti-p200 Pemphigoid. Arch Dermatol. 2011;147(11):1306-10. PubMed PMID: 22106117.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - IgA anti-p200 pemphigoid. AU - Wozniak,Katarzyna, AU - Hashimoto,Takashi, AU - Fukuda,Shunpei, AU - Ohyama,Bungo, AU - Ishii,Norito, AU - Koga,Hiroshi, AU - Dainichi,Teruki, AU - Kowalewski,Cezary, PY - 2011/11/23/entrez PY - 2011/11/23/pubmed PY - 2012/1/10/medline SP - 1306 EP - 10 JF - Archives of dermatology JO - Arch Dermatol VL - 147 IS - 11 N2 - BACKGROUND: Anti-p200 pemphigoid is a rare autoimmune subepidermal blistering disorder. Clinically, it may resemble bullous pemphigoid, linear IgA bullous dermatosis, or dermatitis herpetiformis. Immunologically, anti-p200 pemphigoid is characterized by the development of IgG antibodies directed against a basement membrane zone protein with a molecular weight of 200 kDa. OBSERVATIONS: We report the first case, to our knowledge, of anti-p200 pemphigoid associated with IgA antibodies and having clinical features resembling pemphigus herpetiformis or dermatitis herpetiformis localized on traumatized areas. Histopathological examination of lesional skin showed dermal-epidermal separation and microabscesses composed of neutrophils in the dermal papillae. Direct immunofluorescence disclosed the presence exclusively of linear in vivo-bound IgA along the basement membrane zone. With the use of laser scanning confocal microscopy, in vivo-bound IgA was localized above collagen type IV and colocalized with laminin 332. Indirect immunofluorescence showed circulating IgA antibodies against basement membrane zone at a titer of 1:160 that reacted with the floor of an artificial blister of salt-split skin. Western immunoblot analysis using dermal extract confirmed the reactivity of circulating IgA antibodies with the 200-kDa antigen corresponding to laminin γ1; however, immunoblotting using recombinant protein of 107 amino acid C-terminus of laminin γ1 was negative for circulating IgA antibodies. Immunoelectron microscopy disclosed the reactivity of circulating IgA autoantibodies within the lower lamina lucida. CONCLUSION: To the best of our knowledge, this is the first case fulfilling the immunopathological criteria for anti-p200 pemphigoid associated with IgA antibodies and having unusual clinical features. SN - 1538-3652 UR - https://www.unboundmedicine.com/medline/citation/22106117/IgA_anti_p200_pemphigoid_ L2 - https://jamanetwork.com/journals/jamadermatology/fullarticle/10.1001/archdermatol.2011.303 DB - PRIME DP - Unbound Medicine ER -