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Clinical Chemistry and Laboratory Medicine (CCLM)

Published in Association with the European Federation of Clinical Chemistry and Laboratory Medicine (EFLM)

Editor-in-Chief: Plebani, Mario

Ed. by Gillery, Philippe / Lackner, Karl J. / Lippi, Giuseppe / Melichar, Bohuslav / Payne, Deborah A. / Schlattmann, Peter / Tate, Jillian R.


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1437-4331
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Autoimmune bullous disorders

Rüdiger Eming1 / Michael Hertl2 /

1.

2.

Corresponding author: Dr. med. Rüdiger Eming, Klinik für Dermatologie und Allergologie, Philipps-Universität Marburg, Deutschhausstraße 9, 35033 Marburg, Germany Phone: +49-6421-2866280, Fax: +49-6421-2862902,

Citation Information: Clinical Chemical Laboratory Medicine. Volume 44, Issue 2, Pages 144–149, ISSN (Online) 1437-4331, ISSN (Print) 1434-6621, DOI: 10.1515/CCLM.2006.027, February 2006

Publication History

Published Online:
2006-02-13

Abstract

Bullous skin diseases represent a group of organ-specific autoimmune disorders characterised by binding of circulating autoantibodies to adhesion molecules of the epidermis and the dermo-epidermal basement membrane zone. Binding of these autoantibodies to their antigenic targets results in loss of adhesion between epidermal keratinocytes and at the level of the basement membrane zone. Chronic blisters and secondary painful erosions are the clinical hallmark of autoimmune bullous disorders. Histopathology reveals the location of blister formation and helps to classify the subtype of the bullous skin disorder. Immunofluorescence is crucial for diagnosing autoimmune bullous skin disorders. Tissue-bound autoantibodies are detected by direct immunofluorescence of perilesional skin. Circulating autoantibodies can be visualised by indirect immunofluorescence using tissue substrates such as monkey oesophagus and sodium chloride-split human skin. Most of the autoantigens are available as recombinant proteins, which allows for autoantibody screening by ELISA or immunoblot analysis to confirm the primary diagnosis and, importantly, for immunoserological follow-up of patients.

Keywords: adhesion molecules; autoantibodies; basement membrane zone; bullous skin diseases; desmosomes; ELISA; immunofluorescence; sodium chloride-split skin

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Citing Articles

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[1]
R. Cepeda-Valdés, G. Pohla-Gubo, J.R. Borbolla-Escoboza, O. Barboza-Quintana, J. Ancer-Rodríguez, H. Hintner, and J.C. Salas-Alanisa
Actas Dermo-Sifiliográficas (English Edition), 2010, Volume 101, Number 8, Page 673
[2]
Nina van Beek, Diana Knuth- Rehr, Peter Altmeyer, Chalid Assaf, Philipp Babilas, Christiane Bayerl, Sandrine Benoit, Edgar Dippel, Isaak Effendy, Rüdiger Eming, Matthias Fischer, Thomas Glaenz, Regine Gläser, Matthias Goebeler, Harald Gollnick, Steven Götze, Gerd Gross, Eva Hadaschik, Rudolf Herbst, Barbara Hermes, Bernhard Homey, Nico Hunzelmann, Michael Jünger, Alexander Kapp, Johannes S. Kern, Andreas Körber, Thomas Luger, Dirk Mechtel, Mosaad Megahed, Ingrid Moll, Klaus-Peter Peters, Christiane Pfeiffer, Johannes Ring, Martin Röcken, Miklós Sárdy, Cornelia S. Seitz, Rudolf Stadler, Kerstin Steinbrink, Michael Sticherling, Rolf-Markus Szeimies, Michael Tronnier, Jens Ulrich, Thomas Vogt, Nicola Wagner, Julia Welzel, Jörg Wenzel, Gottfried Wozel, Christos C. Zouboulis, Detlef Zillikens, and Enno Schmidt
JDDG: Journal der Deutschen Dermatologischen Gesellschaft, 2012, Volume 10, Number 7, Page 492
[3]
R. Cepeda-Valdés, G. Pohla-Gubo, J.R. Borbolla-Escoboza, O. Barboza-Quintana, J. Ancer-Rodríguez, H. Hintner, and J.C. Salas-Alanis
Actas Dermo-Sifiliográficas, 2010, Volume 101, Number 8, Page 673
[4]
Minia Campos Domínguez, Ricardo Suárez Fernández, and Pablo Lázaro Ochaita
Actas Dermo-Sifiliográficas, 2006, Volume 97, Number 8, Page 485
[5]
Andrea Kneisel and Michael Hertl
JDDG: Journal der Deutschen Dermatologischen Gesellschaft, 2011, Volume 9, Number 11, Page 927
[6]
Ines Lakoš Jukić and Branka Marinović
Clinics in Dermatology, 2011, Volume 29, Number 4, Page 389
[7]
Silke C. Hofmann, Ocko Kautz, Michael Hertl, Michael Sticherling, Detlef Zillikens, and Leena Bruckner-Tuderman
Journal der Deutschen Dermatologischen Gesellschaft, 2009, Volume 7, Number 3, Page 227
[8]
M. Laimer, C.M. Lanschützer, E. Nischler, A. Klausegger, A. Diem, G. Pohla-Gubo, J.W. Bauer, and H. Hintner
Monatsschrift Kinderheilkunde, 2008, Volume 156, Number 2, Page 110

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