Autoantibodies to bullous pemphigoid antigen 180 induce dermal-epidermal separation in cryosections of human skin

The Journal of Investigative Dermatology
Cassian SitaruD Zillikens

Abstract

Bullous pemphigoid is a subepidermal autoimmune blistering disease associated with autoantibodies to the hemidesmosomal bullous pemphigoid antigens 180 and 230. Most sera from bullous pemphigoid patients recognize epitopes within the N-terminal NC16A portion of the bullous pemphigoid 180 ectodomain. Using cryosections of human skin, patients' sera were shown to generate dermal-epidermal separation when coincubated with leukocytes and complement from healthy volunteers; however, the specificity of pathogenic autoantibodies in bullous pemphigoid patients has not yet been elucidated. In this study, by the use of a modified version of the cryosection model, we show that sera from all of 13 bullous pemphigoid patients and from two rabbits, immunized against bullous pemphigoid 180 NC16A, induced dermal-epidermal separation. This finding was confirmed with the use of IgG purified from patients' sera, whereas sera and purified IgG from healthy controls were not pathogenic. The induction of subepidermal splits in this experimental model was shown to be dependent on the presence of neutrophils, but not complement. Interestingly, patients' autoantibodies affinity purified against a recombinant form of bullous pemphigoid 180 NC16A retained...Continue Reading

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Citations

Feb 6, 2007·Archives of Dermatological Research·Cassian SitaruDetlef Zillikens
Sep 20, 2007·Archives of Dermatological Research·Lisa LeightyZhi Liu
Apr 21, 2005·The Journal of Clinical Investigation·Cassian SitaruDetlef Zillikens
May 4, 2006·The Journal of Clinical Investigation·Michael HertlChristian Veldman
Jan 31, 2013·Orphanet Journal of Rare Diseases·Mircea Teodor ChiriacCassian Sitaru
Aug 23, 2012·PloS One·Katerina VafiaEnno Schmidt
Nov 21, 2015·Expert Review of Clinical Immunology·Nina van BeekEnno Schmidt
Jun 7, 2016·Clinics in Dermatology·Christian D SadikDetlef Zillikens
Oct 6, 2015·The Journal of Investigative Dermatology·Susen MüllerRalf J Ludwig
Dec 15, 2012·Lancet·Enno Schmidt, Detlef Zillikens
Oct 27, 2015·Archives of Dermatological Research·Michael MersmannEnno Schmidt
Feb 12, 2004·The Journal of Investigative Dermatology. Symposium Proceedings·Zhi Liu
May 9, 2012·Immunology and Allergy Clinics of North America·Hideyuki UjiieHiroshi Shimizu
Feb 22, 2012·Immunology Letters·Tamás Németh, Attila Mócsai
Dec 6, 2011·Clinics in Dermatology·Giovanni Di ZenzoLuca Borradori
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Dec 17, 2003·The Journal of Investigative Dermatology·Iakov ShimanovichDetlef Zillikens
Dec 17, 2003·The Journal of Investigative Dermatology·Yoshiaki HirakoDetlef Zillikens
Feb 14, 2004·The Journal of Investigative Dermatology·Giovanni Di ZenzoGiovanna Zambruno
Nov 26, 2002·The Journal of Investigative Dermatology·Zhi Liu
Dec 28, 2007·Advances in Dermatology·Giovanni Di ZenzoLuca Borradori
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Sep 10, 2009·Clinical and Experimental Immunology·A V MarzanoM Cugno
Nov 9, 2005·Experimental Dermatology·Cassian Sitaru, Detlef Zillikens

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