Autoimmune bullous diseases (AIBD) comprise a group of mucocutaneous autoimmune disorders with antigen targets on keratinocytes (pemphigus) or in the epidermal basement membrane zone (EBMZ; pemphigoid diseases). Pemphigus is caused by autoantibodies against either desmoglein 1 and 3, which are transmembrane adhesion molecules with particular function in desmosome contacts between keratinocytes of skin and mucous membranes. The disease is a typical organ-specific autoimmune disease with defined autoantigens to which even Ig-Fab fragments are sufficient to cause disease without the need of additional inflammatory components. Pemphigoid diseases are an AIBD group with subepidermal blistering of which bullous pemphigoid is the most prevalent that starts especially in elderly.
The overarching objectives of our research are to improve clinical and laboratory diagnosis. We have revealed the common existence of nonbullous pemphigoid in nursing homes, delineated the minimal requirements for optimal serologic testing of pemphigoid, and created new serological assays for autoantibody detection in AIBD.
Moreover we examine treatments with new drugs. In the Center for Blistering Diseases we have treated with success large cohorts with anti-CD20 antibodies.
Based on advanced knowledge on B cell pathogenicity in pemphigus, we aim to find more personalized treatments using a strategy of antibody-specific B-cell elimination.
Dept of Dermatology, Center for Blistering Diseases, University Medical Center Groningen, University of Groningen, The Netherlands
Meijer JM, Atefi I, Diercks GFH, Vorobyev A, Zuiderveen J, Meijer HJ, Pas HH, Zillikens D, Schmidt E, Jonkman MF. Serration pattern analysis for differentiating epidermolysis bullosa acquisita from other pemphigoid diseases. J Am Acad Dermatol. 2017.
Romeijn TR, Jonkman MF, Knoppers C, Pas HH, Diercks GF. Complement in bullous pemphigoid: results from a large observational study. Br J Dermatol. 2017.
Giurdanella F, Diercks GF, Jonkman MF, Pas HH. Laboratory diagnosis of pemphigus: direct immunofluorescence remains the gold standard. Br J Dermatol. 2016.
Meijer JM, Lamberts A, Pas HH, Jonkman MF. Significantly higher prevalence of circulating bullous pemphigoid-specific IgG autoantibodies in elderly patients with a nonbullous skin disorder. Br J Dermatol. 2015.
Jonkman MF. Autoimmune Bullous Diseases (2016). Springer International Publishing: Cham. ISBN 978-3-319-23753-4.
E. Sokol: Pemphigus pathogenesis: insights from light and electron microscopic studies. Awarded: 2016
A.M. Poot: Pemphigus: insights in diagnosis and pathogenesis. Awarded: 2016
G. van der Wier: Acantholysis in pemphigus. Awarded: 2014
J.J.A. Buijsrogge: Unusual variants of subepidermal autoimmune blllous diseases. Awarded: 2011
D.A.M. Oktarina: Pemphigus pathogenesis: insights from patient skin studies. Awarded: 2010
Current PhD projects
Joost Meijer: Bullous pemphigoid
Aniek Lamberts: Non-bullous pemphigoid
Federica Giurdanella: Pemphigus