Analysis of the autoimmune response against BP180 and BP230 in ethnic Poles with neurodegenerative disorders and bullous pemphigoid

Abstract recent studies postulated the association between bullous pemphigoid (BP) and neurodegenerative disorders (nD). the autoantibodies to BP180 and/or BP230 may be present not only in BP, but also in nD as neuronal isoforms of these proteins are identified in the central nervous system. however, there are only scant data about the precise pathogenetic mechanisms interlinking nD and BP as well as the immunologic profile in these patients. the aim is to analyze the serological immunopathological profiles (anti-BP180 igG, anti-BP230 igG) in BP patients with and without nD in order to identify the specific autoantibody(ies) and corresponding antigens responsible for nD development in BP patients. altogether, 82 ethnic Poles with BP and their medical records were examined (62 BP-nD; 20 BP+nD). Levels of serum anti-BP180/BP230 igG in BP patients were evaluated with eLisas. the statistical analyses involved Pearson chi-squared test, Mann-whitney u-test and ranking of autoantibodies. the prevalence of nD among BP patients was 24.4%. there were no statistically significant differences in autoantigens profiles (anti-BP180/anti-BP230 igG) between BP+nD and BP-nD groups. there was no relationship between nD development and anti-BP180/anti-BP230 igG leve

Association between Levels of IgA Antibodies to Tissue Transglutaminase and Gliadin-Related Nonapeptides in Dermatitis Herpetiformis

Dermatitis herpetiformis (DH) is an autoimmunity-driven inflammatory blistering dermatosis associated with a gluten-dependent enteropathy. Tissue transglutaminase (tTG) and nonapeptides of gliadin (npG) are considered in its pathomechanism/diagnostics. Here, the diagnostic accuracy of anti-tTG/anti-npG IgA ELISAs in Slavic DH patients with active skin rash was assessed through creating receiver operating characteristic (ROC) curves, determining cutoff values, and calculating correlations between levels of anti-tTG/anti-npG IgA in DH, IgA/neutrophil-mediated non-DH patients and healthy persons. Altogether, sera from 80 Slavic individuals were examined. There were negligible differences between cutoff points obtained by the ELISAs manufacturer and those in this study. There were statistically significant correlations between levels of anti-tTG/anti-npG IgA in both DH group and the group of IgA/neutrophil-mediated non-DH dermatoses. There was no such correlation in healthy controls. It seems that IgA autoantibodies to tTG and npG in the IgA/neutrophil-mediated DH are produced in the coordinated way implying their causal relationship

Immunoexpression of IgA receptors (CD89, CD71) in dermatitis herpetiformis

Introduction. The role of IgA receptors in dermatitis herpetiformis (DH) pathogenesis is still unknown. CD89 and CD71 may be associated with immune response during DH development. The purpose of this study was to perform semiquantitative analysis of simultaneous immunoexpression of CD89 and CD71 in DH and IgA/neutrophil-mediated non-DH dermatoses (IgAN) in relation to specific IgA autoantibodies/antibodies (tissue and epidermal transglutaminases, nonapeptides of gliadin — eTG/tTG/npG) as well neutrophil activation via the release of neutrophil elastase (NE). Material and methods. In total, 48 patients were studied. The study was conducted on skin lesions and sera obtained from DH and IgAN patients. DH and IgAN served as mutually positive control groups. We used immunohistochemical technique with semiquantitative digital morphometry and ELISA to measure serum levels of anti-eTG/tTG/npG IgA. Results. CD89 showed a significantly higher expression in DH than in IgAN. CD71 was overexpressed in DH and IgAN. CD89 immunoexpression correlated negatively with CD71 in IgAN. A positive correlation was revealed between CD89 immunoexpression and anti-npG IgA in DH. No statistically significant correlations were found in DH between the CD89/CD71 and NE immunoexpression, between CD71 immunoexpression and anti-tTG/eTG/npG IgA, or between CD89 immunoexpression and anti-eTG/tTG IgA serum levels. Conclusions. CD89 is probably a key IgA Fc receptor in DH development, where it is associated with immune response to gluten. CD71 may be linked with inflammation in DH and IgAN. We suggest that interaction between CD89 and CD71 can modulate the inflammation in IgAN

Involvement of Nail Apparatus in Pemphigus Vulgaris in Ethnic Poles Is Infrequent

Pemphigus vulgaris lesions have a tendency to localize around natural body orifices. The aim here was to analyze the involvement of nail apparatus in pemphigus vulgaris. Sixty seven ethnic Poles suffering from pemphigus vulgaris on photographic files archiving initial presentation were retrospectively evaluated. Pemphigus vulgaris was diagnosed using combination of clinical data, H+E histology, direct immunofluorescence of plucked scalp hair and/or perilesional tissue also for IgG1 and IgG4 deposits evaluation, indirect immunofluorescence on mosaic substrate and/or monkey esophagus, mono-analyte ELISA with desmoglein 1/3 or multi-analyte ELISA. The nail apparatus involvement was found in 9 of 67 patients (13.4%; 3 females and 6 males). Periungual fingernail lesions were found in 6 patients (2 females, 4 males), whereas periungual toenail lesions in just 3 patients (1 female, 2 males). Our patients nail apparatus changes included, by order of frequency, paronychia, nail discoloration, onychorrhexis, Beau lines, periungual hemorrhages, onychomadesis, cross-ridging, onycholysis, and trachyonychia. The average time between the onset, as recalled by patients, and the diagnosis of pemphigus vulgaris with direct immunofluorescence was not statistically different in PV patients with and without nail apparatus lesions. In this article the molecular and immunological rationale for of periungual involvement is discussed. Our single-center study suggests that nail apparatus involvement is infrequent in pemphigus vulgaris in ethnic Poles. Due to the fact that nail apparatus lesions in pemphigus vulgaris may clinically resemble onychomycosis, giving the proper diagnosis can be difficult particularly when other lesions are overlooked or misinterpreted