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Introduction Patients qualified for the Polish government programme of treating severe pemphigus diseases with rituximab (RTX) available in 2018-2019 had to meet numerous criteria, including no active infectious disease. Aim The clinical usefulness of tuberculosis screening with the QuantiFERON-TB Gold Plus (QFT-Plus) in native pemphigus patients selected for RTX treatment was statistically evaluated. Material and methods Eighteen pemphigus patients were examined with QFT-Plus prior to the intended RTX therapy. Ninety hospital employees examined with QFT-Plus due to contact with a cleaning worker who was diagnosed with active pulmonary tuberculosis were the control group. Results Six of 18 pemphigus patients had a positive QFT-Plus test result, one indefinite result and one initially indefinite and then negative. In the control group, 26 of 90 employees had a positive test result and none had an indefinite result. Statistical analysis by Fisher's exact test showed no statistically significant difference in QFT-Plus positive results between the groups (p = 0.5577). Only in 1 patient with recurrent mucocutaneous pemphigus vulgaris previously treated with traditional immunosuppression, lung changes were detected by computed tomography. No employee had any changes in the chest radiograph. Conclusions Prior immunosuppression and autoimmunity might be the cause of indefinite test results, but they do not seem to increase positive results. In the native population, the QFT-Plus screening reveals a significant population exposure to M. tuberculosis infection independent of pemphigus autoimmunity, and such screening can be a starting point for identifying patients requiring anti-tuberculosis drug prophylaxis before combined RTX-glucocorticosteroid treatment.

Background The current standard in the serologic diagnosis of autoimmune bullous diseases (AIBD) is a multistep procedure sequentially applying different assays. In contrast, the BIOCHIP Mosaic technology combines multiple substrates for parallel analysis by indirect immunofluorescence. Methods Sera from 749 consecutive, prospectively recruited patients with direct immunofluorescence–positive AIBD from 13 international study centers were analyzed independently and blinded by using (1) a BIOCHIP Mosaic including primate esophagus, salt-split skin, rat bladder, monkey liver, monkey liver with serosa, recombinant BP180 NC16A, and gliadin GAF3X, as well as HEK293 cells expressing recombinant desmoglein 1, desmoglein 3, type VII collagen, and BP230 C-terminus and (2) the conventional multistep approach of the Department of Dermatology, University of Lubeck. Results In 731 of 749 sera (97.6%), specific autoantibodies could be detected with the BIOCHIP Mosaic, similar to the conventional procedure (725 cases, 96.8%). The Cohen κ for both serologic approaches ranged from 0.84 to 1.00. In 6.5% of sera, differences between the 2 approaches occurred and were mainly attributed to autoantigen fragments not present on the BIOCHIP Mosaic. Limitations Laminin 332 and laminin γ1 are not represented on the BIOCHIP Mosaic. Conclusions The BIOCHIP Mosaic is a standardized time- and serum-saving approach that further facilitates the serologic diagnosis of AIBD.

Background: Pemphigus encompasses a group of life-threatening autoimmune bullous diseases characterized by blisters and erosions of the mucous membranes and skin. Before the era of immunosuppressive treatment, the prognosis of pemphigus was almost always fatal. Due to its rarity, only few randomized controlled therapeutic trials are available. Recently, rituximab has been approved as first-line treatment for moderate and severe pemphigus vulgaris in Europe and the USA.Objectives: The Autoimmune blistering diseases Task Force of the European Academy of Dermatology and Venereology (EADV) has initiated a throughout update of the guideline for the management of patients with pemphigus. Results: The guidelines for the management of pemphigus were updated and the degree of consent among all task force members was included. The final version of the guideline was consented by the European Dermatology Forum (EDF) and several patient organisations.

Justyna Gornowicz-Porowska,1 Magdalena Jałowska,2 Agnieszka Seraszek-Jaros,3 Monika Bowszyc-Dmochowska,4 Elżbieta Kaczmarek,3 Marian Dmochowski2 1Department and Division of Practical Cosmetology and Skin Diseases Prophylaxis, Poznan University of Medical Sciences, Poznan, Poland; 2Autoimmune Blistering Dermatoses Section, Department of Dermatology, Poznan University of Medical Sciences, Poznan, Poland; 3Department of Bioinformatics and Computational Biology, Poznan University of Medical Sciences, Poznan, Poland; 4Cutaneous Histopathology and Immunopathology Section, Department of Dermatology, Poznan University of Medical Sciences, Poznan, PolandCorrespondence: Marian Dmochowski, Autoimmune Blistering Dermatoses Section, Department of Dermatology, Poznan University of Medical Sciences, Poznan, Poland, Tel +48 61 8691319, Email mdmochowski@ump.edu.plPurpose: Mucous membrane pemphigoid (MMP) is a very rare autoimmune bullous disease, affecting predominantly the mucosae and characterized by autoantibodies to the epithelial basement membrane components. Laminin 332 (Ln-332) is one of the most probable antigens with association with malignancy. The laboratory diagnosis of Ln-332-mediated autoimmunity is troublesome. The aim here was to comparatively examine IgG, IgG4, and IgA autoantibodies specific to α 3, β 3 or γ 2 subunits of Ln-322 in MMP patients using the BIOCHIP mosaic-based indirect immunofluorescence technique (IIF).Patients and Methods: Sera from 15 MMP patients were studied. BIOCHIP mosaic-based Ln-332 IIF, direct immunofluorescence, ELISA tests for anti-BP180/BP20 IgG antibodies and statistical analyses were performed.Results: Of all the 15 sera examined for IgG4 antibodies, only 1 (6.67%) reacted with the α 3 chain, 0 with the β 3 chain, and 0 with the γ 2 chain. No positive reactivity was seen with the IgG and IgA antibodies. BIOCHIP mosaic-based IIF with Ln-332 showed 100% sensitivity, 8% specificity, 21% positive predictive value, and 100% negative predictive value in relation to the diagnostic gold standard of DIF. The concomitant malignancies were revealed in three cases.Conclusion: The detection of antibodies to Ln-332 chains is occasional in Polish MMP sufferers. Still, the evaluation of IgG4 antibodies in MMP can reduce the false-negative results.Keywords: mucous membrane pemphigoid, laminin 332, immunologic test

Bullous pemphigoid (BP) is a cutaneous disease triggered by numerous stimuli, where genetic milieu-influenced autoimmunity to hemidesmosomal proteins, namely, BP180 and/or BP230 initiate an inflammation leading to dermal-epidermal junction (DEJ) enzymatic pathological remodelling. Here, to the best of our knowledge, we present the first case of an infantile BP apparently triggered by COVID-19. BP should be included in differential diagnosis of infantile rashes showing blisters or vesicles or both as well as their prodromal and evolutionary lesions. Possible triggers, such as coronavirus disease 2019 (COVID-19), of BP in infancy should be identified and properly dealt with.

Immune responses to tissue transglutaminase (tTG) and nonapeptides of gliadin (npG) are associated with dermatitis herpetiformis (DH), a gluten-related dermatosis. Recently, a bi-analyte immunoblot (b-aIB) was introduced to detect IgA antibodies in response to tTG and npG. We compared the utility of ELISA and b-aIB with tTG in serological diagnoses of DH and their agreement with direct immunofluorescence (DIF). In total, 55 sera (27 DIF-positive DH patients, 4 DIF-negative DH patients and 24 healthy controls) were examined. ELISA for anti-tTG IgA, b-aIB for anti-npG and anti-tTG IgA, and statistical analysis were performed. The b-aIB with tTG showed 78% sensitivity, 100% specificity, 100% positive predictive value, and 82% negative predictive value in relation to ELISA. A better rate of agreement (Cohen’s kappa values) in IgA detection was observed in the pair tTG ELISA and b-aIB with npG (0.85) than in pairs tTG ELISA and b-aIB with tTG (0.78) or b-aIB with tTG and b-aIB with npG (0.78). No degree of agreement was found between serological tests and DIF. Both serological tests may be used to detect the anti-tTG IgA in DH patients. Still, DH diagnosing requires careful consideration of clinical data as well as results of tissue imaging (crucial DIF) and immunoserological techniques detecting DH-type features.

This mini-review presents an update on the direct immunofluorescence (DIF) for diagnosing dermatitis herpetiformis. The DIF of uninvolved, perilesional skin is a crucial laboratory procedure in diagnosing dermatitis herpetiformis (DH). IgA deposits at the dermal-epidermal junction (DEJ) of perilesional skin with DIF can also be found in coeliac patients with inflammatory skin diseases different from DH. In certain patients presenting with the rash resembling DH, the deposition of exclusively C3 at DEJ can be found. The term "granular C3 dermatosis" was proposed to name such a rash. Recent data on DH suggest that perhaps the very concept of DH that we are universally accepting now is misleading and should be revised.

Introduction Dermatitis herpetiformis (DH) is a chronic, pruritic, gluten-induced skin disorder characterized by subepidermal granular IgA deposition and a variable degree of enteropathy identical to that seen in coeliac disease. So far, there has been no European consensus about the management of DH. Methods The guidelines were created by small subgroups of a guideline committee consisting of 26 specialists from various medical fields and one patients' representative. The members of the committee then discussed the guidelines and voted for the final version at two consensus meetings. The guidelines were developed under the support of the European Academy of Dermatology and Venereology (EADV) and in collaboration with the European Dermatology Forum (EDF). Results The guidelines summarize evidence-based and expert-based recommendations (S2 level) for the management of DH (see Appendix). Conclusion These guidelines will improve the quality of management of DH and support dermatologists in their diagnostic and therapeutic decisions.

In this paper, we present our own clinical-laboratory experience concerning three less obvious presentations of pemphigus vulgaris (PV) and discuss the pertinent literature. The involvement of the sacral dimple reported here for the first time, as well as the nipple and the eyes, could initially be misleading clinically. These less stereotypical localizations may occur due to the transition of different epithelia, each with varying levels of cadherin (desmoglein, desmocollin) and thus altered sensitivity to mechanical stress. The role of dermatologists who have experience in treating autoimmune blistering dermatoses is fundamental for identifying promptly the initial and exacerbating PV lesions in such unusual locations.

Autoimmune bullous diseases are potentially life-threatening dermatoses which present with cutaneous and/or mucosal blisters, diagnosed on the basis of clinical manifestations, direct immunofluorescence of perilesional tissue, and serum testing for circulating autoantibodies. Sometimes, lesions in the navel can lead to the diagnosis of a bullous disease.To assess the frequency of occurrence of pemphigus lesions located in the navel area and nail apparatus in pemphigus vulgaris (PV) in ethnic Poles.Eighty one patients (31 males and 50 females, mean age 59 years) with dermatoses of the PV group diagnosed in 2002-2020 were retrospectively analysed using their photographic files. Statistical analysis was performed using the difference test between two proportions to check the difference between the percentage of PV patients with navel area involvement and nail apparatus involvement.There was no statistically significant difference between PV patients with nail apparatus involvement (12.3%) and navel area involvement (14.8%) (It is speculated that the causal relationship may exist between the female reproductive system and the pattern of expression of PV lesions around the umbilicus. The awareness that PV can infrequently affect the umbilical region and the nail apparatus should help in some cases to establish the diagnosis of PV. The periumbilical involvement can facilitate the performance of DIF in individuals with lesions in less accessible areas.

Introduction Autoimmune bullous diseases (ABDs) are potentially life-threatening mucocutaneous illnesses that require diagnosis with direct immunofluorescence (DIF). In this study we compared the diagnostic accuracy of traditional DIF (DIFt; separate immunoglobulin (Ig) G, IgG1, IgG4, IgA, IgM and C3 deposits detection) and modified DIF (DIFm; simultaneous IgG + IgG4 deposits detection instead of separate IgG and IgG4 deposits detection) in routine diagnostics of ABDs. Material and methods Eighteen patients with ABDs (7 with pemphigus dermatoses and 11 with subepithelial ABDs) were evaluated with DIFt and DIFm. Results The agreement of detectability of IgG immunoreactants was obtained in 16 ABD cases (88.89%), as positive results in both DIFt and DIFm were obtained in 13 cases and negative results in both DIFt and DIFm were obtained in 3 cases. One ABD case (Brunsting-Perry pemphigoid) (5.56%) was negative in DIFm with a positive DIFt result (IgG1 deposits). One ABD case (bullous pemphigoid) (5.56%) had only C3 deposits in DIFt with a positive DIFm reading (IgG + IgG4 deposits). A statistically significant relationship (p = 0.0186) between DIFm and DIFt results was revealed using Fisher's exact test. Conclusions Both DIFt and DIFm are useful methods to detect deposition of IgG immunoreactants, but it seems that the innovative DIFm method slightly increases the detectability of IgG/IgG4 immunoreactants in relation to DIFt. The introduction of DIFm into routine laboratory diagnostics of ABDs seems to be justified, as it enables the abandonment of separate FITC conjugates for IgG and IgG4, which is important for cost-effectiveness.

Autoimmune blistering dermatoses (ABDs) are characterized by autoantibodies to keratinocyte surface antigens and molecules within the dermal–epidermal junction causing disruption of skin integrity. The affinity of Fc receptors (FcRs) causing an autoimmune response in ABDs may vary based on single-nucleotide polymorphisms (SNPs) in FcRs determining the course of disease. This study aimed to explore the effects of CD16A and CD32A SNPs on the autoimmune response in several ABDs. In total, 61 ABDs patients were investigated. ELISA tests, direct immunofluorescence (DIF), TaqMan SNP Genotyping Assays, and statistical analyses were performed. The CA genotype (composed of allele C and A) of rs396991 in CD16A had a higher affinity for tissue-bound IgG1 in pemphigus and for C3 in subepithelial ABDs, showing statistical significance. The greatest relative risk (odds ratio) was reported for AA (rs396991 of CD16A) and CC (rs1801274 of CD32A) homozygotes. There were no statistically significant differences between certain genotypes and specific circulating autoantibodies (anti-DSG1, anti-DSG3 IgG in pemphigus; anti-BP180, anti-BP230 IgG) in subepithelial ABDs. Our findings indicated that rs396991 in CD16A may be of greater importance in ABDs development. Moreover, FcR polymorphisms appeared to have a greater impact on tissue-bound antibodies detected using DIF than circulating serum antibodies in ABDs.

Novel appearances in cutaneous pathology as well as mucocutaneous clinical signs are being described which indicate that this is still an attractive area for exploration. The H + E histology terms of “decorated tomb stoning” and “undecorated tomb stoning”, advocated by some pathologists, are misleading and as such should be avoided. Here, an appearance of IgG4 pemphigus deposits examined cost-effectively with direct immunofluorescence and suggested to be called “dew drops on spider web” is depicted in depth.

A range of pemphigus is relatively rare potentially fatal group of autoimmune blistering dermatoses. Usually, there is no apparent triggering, while in some predisposed patients there are alleged environmental/industrial inducing factors. In a short time period (4 years), we diagnosed 3 novel cases of pemphigus (1 pemphigus vulgaris, 1 pemphigus foliaceus and 1 shift from pemphigus foliaceus into pemphigus vulgaris) at a clinical and laboratory level (ELISA, immunofluorescence studies). We discuss a possible common inducing mechanism as these patients inhabit one estate of the Poznan suburbia (Kozieglowy, population < 12,000), Greater Poland district, Poland, and review literature data on alleged pemphigus triggers. To the best of our knowledge, this is the first report exploring the putative association between pemphigus diseases and wastewater treatment plant waterborne or volatile by-products in the vicinity of such a facility.

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 level (p = 0.5933, p = 0.4701, respectively). The autoantibodies levels of BP+ND and BP-ND patients show insignificant differences suggesting that also in ethnic Poles a hypothetical pathogenetic association of BP and ND, but not only an aging-related epidemiological one, appears to be independent of a particular BP antigen. Nevertheless, it cannot be excluded that phenomena of epitopes spreading, immune cross-reaction and conformational changes in BP180/BP230 may underlie BP development in ND patients.

Introduction : Pemphigus and bullous pemphigoid (BP) are identified by autoantibodies (abs) against desmoglein 1, 3 (DSG1/3) and BP180/BP230, respectively. A novel mosaic to indirect immunofluorescence (IIF) using purified BP180 recombinant proteins spotted on slide and transfected cells expressing BP230, DSG1, DSG3 is available. The commercial (IgG detection) and modified (IgG4 detection) mosaic for indirect immunofluorescence (IIFc – IIF commercial, IIFm – IIF modified) and IgG ELISAs were evaluated in pemphigus and bullous pemphigoid (BP) molecular diagnostics. Aim : To compare diagnostic accuracy of commercial (IgG detection) and modified (IgG4 detection) mosaic IIF assay and to examine the diagnostic value of ELISAs in relation to mosaic IIF in routine laboratory diagnostics of pemphigus and BP. Material and methods : Sera from 37 BP and 19 pemphigus patients were studied. Associations between tests were assessed using Fisher’s exact test. Results: There are associations between the positive/negative samples detected by IIFc with desmoglein1 (DSG1)/desmoglein3 (DSG3)/BP230 transfected cells and ELISAs and no association between anti-BP180 IgG detection by IIFc and ELISA. IIFm with DSG1 and DSG3 showed both 100% sensitivity and 100% and 78% specificity, respectively, and 100% and 83% positive predictive value in relation to IIFc. IIFm with BP230 had 87% specificity, 55% sensitivity, whereas IIFm with BP180 had a 100% sensitivity and 13% specificity in relation to IIFc. Conclusions : The IIFc with DSG1/DSG3/BP230 transfected cells, excluding BP180 spots, is an alternative method to ELISA in pemphigus/BP diagnostics. IgG4 antibodies, both pathogenically and diagnostically important, are inconsistently detectable with IIFm.

Here we investigated the cutaneous CD32A and CD89 expression in relation to the neutrophil elastase (NE) expression and serum level of anti-desmoglein 1 and 3 (DSG1/DSG3) IgG in pemphigus, anti-BP180/BP230 IgG in bullous pemphigoid (BP), anti-gliadin nonapeptides (npG), tissue (tTG), and epidermal transglutaminases (eTG) IgA in dermatitis herpetiformis (DH). The examined material consisted of skin/mucosal tissues and sera. In total, 87 patients were studied. Immunohistochemistry on paraffin-embedded sections with quantitative digital morphometry was used to measure the intensity of CD32A/CD89/NE expressions. Levels of anti-DSG1/DSG3 IgG, anti-BP180/BP230 IgG, and anti-npG/tTG/eTG IgA were evaluated with ELISAs. CD32A was abundantly expressed in cutaneous lesions in pemphigus and BP. We found no statistically significant correlation between the CD32A/CD89 and NE expression intensities in pemphigus, BP, and DH. There was a significant correlation between CD89 expression and anti-npG IgA in DH. Our results revealed a lack of correlation between CD32A expressions and anti-DSG1/DSG3 IgG levels in pemphigus, anti-BP180/BP230 IgG in BP as well as CD89 expression and anti-tTG/eTG IgA in DH. CD89 seems to be linked with gluten intolerance in DH rather than with proteolytic destruction of dermal-epidermal junction. CD32A appears to play an important role in mediating skin injury in pemphigus and BP, but probably independently from specific autoantibodies.

Dermatitis herpetiformis (Duhring’s disease) is an autoimmune blistering dermatosis with a characteristic polymorphic, itchy rash. The autoimmune process against transglutaminases (TGs) is connected with asymptomatic or with mild symptoms of gluten-sensitive enteropathy (GSE). The diagnostic approach in DH should rely on clinical evaluation, direct immunofluorescence of perilesional skin and evaluation of serum IgA antibodies with ELISA with one substrate of four substrates from which to choose (tTG, eTG, npG, neo-tTG) using the ELISA method. In cases showing an equivocal clinical-laboratory picture such an approach should be broadened by performing histopathology of lesional skin and examining the HLA DQ2/DQ8 haplotype. Combined treatment with dapsone and a gluten-free diet in individualized combinations is generally the therapeutic method of choice in DH.

Autoimmune blistering dermatoses (ABDs) are characterized by autoantibodies to keratinocyte surface antigens and molecules within the dermal&ndash, epidermal junction causing disruption of skin integrity. The affinity of Fc receptors (FcRs) causing an autoimmune response in ABDs may vary based on single-nucleotide polymorphisms (SNPs) in FcRs determining the course of disease. This study aimed to explore the effects of CD16A and CD32A SNPs on the autoimmune response in several ABDs. In total, 61 ABDs patients were investigated. ELISA tests, direct immunofluorescence (DIF), TaqMan SNP Genotyping Assays, and statistical analyses were performed. The CA genotype (composed of allele C and A) of rs396991 in CD16A had a higher affinity for tissue-bound IgG1 in pemphigus and for C3 in subepithelial ABDs, showing statistical significance. The greatest relative risk (odds ratio) was reported for AA (rs396991 of CD16A) and CC (rs1801274 of CD32A) homozygotes. There were no statistically significant differences between certain genotypes and specific circulating autoantibodies (anti-DSG1, anti-DSG3 IgG in pemphigus, anti-BP180, anti-BP230 IgG) in subepithelial ABDs. Our findings indicated that rs396991 in CD16A may be of greater importance in ABDs development. Moreover, FcR polymorphisms appeared to have a greater impact on tissue-bound antibodies detected using DIF than circulating serum antibodies in ABDs.

Direct immunofluorescence (DIF) microscopy still is the gold standard in diagnosing and differentiating subepidermal autoimmune blistering diseases (SABDs) from other bullous diseases. New optical systems were developed that aim to facilitate DIF images evaluation. The aim of the study was to evaluate the usefulness of three fluorescence microscopy systems with different light source for routine diagnostics of SABDs with DIF. In total, perilesional tissue samples for DIF from 34 SABD patients were examined under the three commercial microscopy systems (short arc mercury lamp-operated microscopy-MLM, blue light-emitting diode technology-operated microscopy-bLED and laser scanning confocal microscopy-LSCM) for the detection and pattern analysis of IgA, IgG, IgG1, IgG4, C3 immunoreactants along the dermal-epidermal junction (DEJ) by three independent observers simultaneously. MLM, bLED, and LSCM provided comparable quality of disease-characterizing immunoreactants imaging (number of immunoreactant types detected and patterns of their deposition along DEJ were the same) but screening of slides was quicker using bLED than both LSCM and MLM, as statistical analysis indicated. It is concluded that bLED is an efficient and preferable system for routinely diagnosing SABDs cost-effectively. RESEARCH HIGHLIGHTS: New optical systems were developed that aim to facilitate direct immunofluorescence evaluation. Here, we evaluate the usefulness of three fluorescence microscopy systems with different light source for routine diagnostics concluding that that bLED is an efficient and preferable system.

Bullous pemphigoid (BP) is an autoimmune blistering dermatosis of the elderly mediated by IgG and IgE antibodies to skin hemidesmosomal proteins, BP180 and/or BP230, that occur physiologically also in neuronal tissue. It was reported that BP is associated with neurodegenerative diseases (ND). We performed a retrospective study in a setting of a Central European university dermatology department on prevalence of ND in 94 BP patients. 26 out of 94 BP patients had at least one ND. ND included: Parkinson’s disease, dementia, stroke, hear loss, tinnitus, blindness, vertigo, neurosyphilis, systemic sclerosis, and epilepsy. Since population aging is conceivably responsible for the rising number of BP cases as a result of immunosenescence-related phenomena, the plausible BP-specific immunopathogenetic relationship between BP and ND deserves to be further experimentally explored.

Background Technical innovation of autoimmune blistering dermatoses (ABDs) diagnosis aimed at multiplex approach. Two multiparametric ELISA tests are commercially available for ABDs serology. The aim was to compare diagnostic accuracy of multiparametric and monospecific ELISAs and to examine the diagnostic value/agreement of multivariant ELISA in compliance with traditional diagnostic setup for ABDs. Methods In total, 128 sera from suspected ABDs patients were studied (27 sera in order to compare ELISAs). Multivariant ELISA (detection of IgG against desmoglein 1 and 3 - DSG1/3; BP180, BP230, envoplakin, type VII collagen), monovariant ELISA, and statistical analysis were performed. Results With the use of sera from patients with suspected ABDs, the multiparametric ELISA yield an agreement of 84% with traditional stepwise diagnostics. Multivariant ELISA with BP180 and BP230 showed 87.5% and 80% sensitivity, 87.5% and 91% specificity, 87.5% reliability as well as 87.5% and 80% positive predictive value, 87.5% and 91% negative predictive value, respectively, in relation to monospecific ELISA. Multivariant ELISA with DSG1 and DSG3 showed 50% and 80% sensitivity, 100% and 80% specificity, 85% and 80% reliability as well as 100% and 57% positive predictive value, 82% and 92% negative predictive value, respectively, in relation to monospecific ELISA. A better rate of agreement was observed among ELISA systems with BP180 and BP230, than with ELISA systems with DSG1 and DSG3. Conclusion Multivariant ELISA test combined with clinical examinations and DIF is recommended as a minimal approach to diagnosing ABDs in ethnic Slavs.

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.

Summary Background Basal cell carcinoma (BCC) is the most common human cancer. It is thought that skewed expression of desmogleins (Dsgs) in BCC may promote tumourigenesis. Aim To comparatively examine expression of Dsg2/Dsg3, using fluorescent in situ hybridization (FISH) and immunohistochemistry (IHC) in BCC subtypes. Methods In total, 84 frozen sections from patients with various clinical or histological subtypes of BCC were analyzed. Expressions of Dsg2/Dsg3 protein and Dsg2/Dsg3 mRNA were evaluated using IHC and FISH, respectively, in BCC nests and BCC-free epidermis, and then quantitatively measured. Results There was loss of correlation between Dsg2 and Dsg3 (IHC) in nodular and superficial BCC (nBCC, sBCC), and significant correlation between Dsg2 and Dsg3 (FISH) in BCC, but not nBCC and sBCC. Conclusions Because more prominent aberrations of Dsg2/Dsg3 expression were seen at the protein than at the mRNA level in BCC, these comparative observations indicate greater importance of events at the proteome level than those at the genome level in tumour functional compartments. Different Dsg2/Dsg3 expression in sBCC and nBCC might corroborate the possibility that sBCC and nBCC are separate conditions. These results may contribute to better understanding of the biological behaviour of BCC.

Pemphigus is an acantholytic bullous disease with a potentially fatal course. The laboratory diagnosis of pemphigus is based on direct immunofluorescence showing the presence of the in vivo bound IgG in intercellular spaces of the epidermis in skin biopsy. For determining the clinical subtypes of pemphigus serum immunological studies allowing characterization of the target antigen(s) are obligatory. Treatment of pemphigus with prednisone at an initial dose of 1–1.5 mg/kg in combination with azathioprine is currently recommended by most dermatologists as well as by the European Expert Group as the therapy of choice in typical cases, whereas other immunosuppressive agents, biological drugs, and intravenous immunoglobulins should be considered in refractory cases and rare subsets of pemphigus.