Etablierung eines ELISAs zum Nachweis von Autoantikörpern gegen Desmocollin 1, 2 und 3 in Seren von Patienten mit Pemphigus-Erkrankung

Bullous autoimmune dermatoses of the pemphigus group are characterized by autoantibodies targeting desmosomal cadherins, i.e. desmogleins (Dsg) and desmocollins (Dsc). Dsg1 and Dsg3 are important autoantigens of pemphigus vulgaris (PV). The aim of this study was to establish an ELISA to examine the prevalence of autoantibodies targeting Dsc. A cohort of 82 PV patients, five IgA pemphigus patients, three patients with paraneoplastic pemphigus (PNP), two patients with atypical pemphigus and 32 so far not further classified patients with bullous dermatoses were examined, as well as 22 healthy control volunteers. One of the patients with atypical pemphigus showed IgG and IgA reactivity against Dsc1, the second patient with atypical pemphigus showed IgA reactivity against Dsc1 and Dsc3 and weak IgA reactivity against Dsc2. One patient with IgA pemphigus was IgA positive against Dsg1, Dsc1 and Dsc2, and one patient with PNP was IgG positive against Dsc3. In contrast, none of the PV patients showed any reactivity against Dsc, and all of the patients with so far unclassified bullous dermatoses were also negative against Dsc. Consequently, Dsc do not represent frequent autoantigens of pemphigus vulgaris, but can be detected as autoantigens in rare types of pemphigus (i.e. IgA pemphigus and PNP) and atypical pemphigus.