TY  - JOUR
A1  - Recke, Andreas
A1  - Konitzer, Sarah
A1  - Lemcke, Susanne
A1  - Freitag, Miriam
A1  - Sommer, Nele Maxi
A1  - Abdelhady, Mohammad
A1  - Amoli, Mahsa M.
A1  - Benoit, Sandrine
A1  - El-Chennawy, Farha
A1  - Eldarouti, Mohammad
A1  - Eming, Rüdiger
A1  - Gläser, Regine
A1  - Günther, Claudia
A1  - Hadaschik, Eva
A1  - Homey, Bernhard
A1  - Lieb, Wolfgang
A1  - Peitsch, Wiebke K.
A1  - Pföhler, Claudia
A1  - Robati, Reza M.
A1  - Saeedi, Marjan
A1  - Sárdy, Miklós
A1  - Sticherling, Michael
A1  - Uzun, Soner
A1  - Worm, Margitta
A1  - Zillikens, Detlef
A1  - Ibrahim, Saleh
A1  - Vidarsson, Gestur
A1  - Schmidt, Enno
T1  - The p.Arg435His Variation of IgG3 With High Affinity to FcRn Is Associated With Susceptibility for Pemphigus Vulgaris-Analysis of Four Different Ethnic Cohorts
JF  - frontiers in Immunology
N2  - IgG3 is the IgG subclass with the strongest effector functions among all four IgG subclasses and the highest degree of allelic variability among all constant immunoglobulin genes. Due to its genetic position, IgG3 is often the first isotype an antibody switches to before IgG1 or IgG4. Compared with the other IgG subclasses, it has a reduced half-life which is probably connected to a decreased affinity to the neonatal Fc receptor (FcRn). However, a few allelic variants harbor an amino acid replacement of His435 to Arg that reverts the half-life of the resulting IgG3 to the same level as the other IgG subclasses. Because of its functional impact, we hypothesized that the p.Arg435His variation could be associated with susceptibility to autoantibody-mediated diseases like pemphigus vulgaris (PV) and bullous pemphigoid (BP). Using a set of samples from German, Turkish, Egyptian, and Iranian patients and controls, we were able to demonstrate a genetic association of the p.Arg435His variation with PV risk, but not with BP risk. Our results suggest a hitherto unknown role for the function of IgG3 in the pathogenesis of PV.
KW  - immunology
KW  - dermatology
KW  - autoantibodies
KW  - allotype
KW  - pemphigus
KW  - Diagnose
KW  - pemphigoid
KW  - half-life
KW  - functional genetics
Y1  - 2018
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-225073
VL  - 9
ER  - 
TY  - JOUR
A1  - Benoit, Sandrine
A1  - Scheurlen, Michael
A1  - Goebeler, Matthias
A1  - Stoevesandt, Johanna
T1  - Structured diagnostic approach and risk assessment in mucous membrane pemphigoid with oesophageal involvement
JF  - Acta Dermato-Venereologica
N2  - Oesophageal involvement in mucous membrane pemphigoid is considered rare, but it may be underdiagnosed. To assess the incidence of oesophageal involvement in a group of patients with newly diagnosed mucous membrane pemphigoid we retrospectively analysed the medical records of 30 consecutive patients with mucous membrane pemphigoid diagnosed between 2006 and 2016 at the Department of Dermatology, University Hospital Würzburg. Twenty-one patients (70%) reported symptoms indicative of oesophageal mucous membrane pemphigoid. Twelve patients (40%) underwent oesophagogastroduodenoscopy, and oesophageal pathology compatible with mucous membrane pemphigoid was endoscopically found in 9 cases (30%). In all patients indirect and direct immunofluorescence were performed. Patients with and without oesophageal involvement did not differ with regard to the results of indirect immunofluorescence on salt-split human skin and monkey oesophagus. Study results demonstrate the necessity of a standardized diagnostic work-up, including adequate tissue samples for direct immunofluorescence, to prevent underdiagnosis of oesophageal mucous membrane pemphigoid.
KW  - cicatricial pemphigoid
KW  - mucous membrane pemphigoid
KW  - oesophagogastroduodenoscopy
KW  - laminin 332
Y1  - 2018
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-176191
VL  - 98
ER  -