Diagnosis Of Autoimmune Bullous Diseases Research Articles

Retrospective analysis of autoimmune bullous diseases in Middle Franconia.

Autoimmune bullous diseases (AIBDs) are a group of rare cutaneous disorders affecting cornified skin and mucous membranes. They are characterized by tense or flaccid blistering and erosions due to autoantibodies against desmosomal and hemidesmosomal structural proteins of the skin. This group of disorders can be divided into those of pemphigoid and those of pemphigus diseases. If left untreated, these autoimmune diseases can cause serious or even life-threatening complications such as loss of fluid, superinfections or impaired food intake. Due to modern standardized serological assays, the diagnosis of AIBDs can usually be confirmed in combination with their clinical appearance. Whereas for a long time corticosteroids were the major players in the treatment of these diseases, with the approval of rituximab and other immunosuppressive agents, the therapy has increasingly improved. In this study, we aimed to investigate epidemiologic and clinical features as well as diagnostics and therapy of bullous autoimmune diseases in Middle Franconia, a governorate within the German federal state of Bavaria. Patients diagnosed or treated because of a AIBDs between 01.04.2013 and 31.03.2019 at the dermatological department of the university hospital Erlangen were included in this retrospective study (n = 242). Patients were either diagnosed for the first time (n=176) or the diagnosis has been confirmed (n=66) at the department. The respective incidence was calculated among the 176 subjects who had been diagnosed at the center in this period. Data was taken from patient records and analyzed with Microsoft® Excel. The evaluation included the diagnoses of pemphigus vulgaris (PV), pemphigus foliaceus (PF), bullous pemphigoid (BP), mucous membrane pemphigoid (MMP), linear IgA dermatosis (LAD), epidermolysis bullosa acquisita (EBA), and dermatitis herpetiformis (DH). This study shows that the incidence of each AIBDs in Middle Franconia is low and comparable (PV, PF, LAD, EBA) or lower (BP, MMP, DH) than in other studies and regions. BP is the most common newly diagnosed AIBD in Middle Franconia. Due to the chronic and sometimes severe course of AIBDs, repeated in-house treatments are often necessary. To date, mainly topically and systemically applied corticosteroids in combination with immunomodulators are used as first-line therapy.

Immunohistochemical Evaluation of C4d and C3d Markers in Bullous Pemphigoid as a Substitute for Direct Immunofluorescence Technique

Background: There are several methods for the diagnosis of autoimmune bullous disease. Direct immunofluorescent (DIF) testing is an important diagnostic method in the diagnosis of immunobullous disease but requires skilled pathologist, fresh tissue and well-equipped laboratory to perform the procedure. The immunohistochemistry analysis of C4d and C3d is easily compared with other methods. This study was conducted to assess the value of immunohistochemistry (IHC) analysis for expressions of C3d and C4d in the diagnosis of bullous pemphigoid (BP). Aims and Objectives: This study was conducted to assess the value of immunohistochemistry (IHC) analysis for expressions of C3d and C4d in the diagnosis of bullous pemphigoid (BP). Materials and Method: We applied C4d and C3d immunohistochemistry on formalin-fixed, paraffin-embedded tissue on 30 cases of bullous pemphigoid that was confirmed by direct immunofluorescence (DIF) evaluation as well as 16 cases in control group (12 cases of herpetiform dermatitis, 3 cases of linear IgA dermatosis and 3 cases of bullous lichen planus). Results: Mean and SD of age were 68.13 ± 14.00, female to male ratio was 1:3. In cases where both C3d and C4d staining were positive, the intensity of C3d staining was higher than C4d. Twenty-two cases showed C4d-positive staining in IHC study, such that in seven cases focal staining and in 15 cases diffuse staining were observed. Also 26 cases showed C3d-positive staining in IHC study such that in four cases focal staining and in 22 cases diffuse staining were observed. In cases with C3d-positive staining, there were 21 cases of deposition only on the bullous floor, one case on the bullous roof and four cases on the bullous roof and floor. In cases with C4d-positive staining, there were 17 cases of deposition on the bullous floor, two cases only on the bullous roof and three cases on the roof and floor. All control cases were negative for C3d and C4d staining in the dermoepidermal junction. For C3d immunohistochemical staining, sensitivity, specificity, positive predictive value and negative predictive value were 86.66%, 100%, 100% and 80%, respectively, and for C4d immunohistochemical staining, respectively, were 73.3%, 100%, 100% and 66.66%. Conclusion: The immunohistochemical specificity of C4d and C3d on tissue blocks is the same as that of direct immunofluorescence test on fresh tissue, but it is less sensitive, so positive results for C3d and C4d immunohistochemical staining on paraffin blocks can be used to confirm the diagnosis of bullous pemphigoid.

Dermoscopic approach for differential diagnosing of autoimmune bullous disease: pemphigus vulgaris, pemphigus foliaceus, and IgA pemphigus.

Dermoscopy is a noninvasive technique for the evaluation of different pigments and microstructures of the epidermis, dermoepidermal junction, and papillary dermis that are not apparent to the naked eye, which therefore improves diagnostic accuracy. This study aims to describe the characteristic dermoscopic features of bullous diseases and analyze the characteristic dermoscopic features of bullous diseases of the skin and hair. A descriptive study was conducted to describe and analyze the characteristic dermoscopic features of bullous diseases in the Zagazig University Hospitals. This study enrolled 22 patients. Dermoscopy revealed yellow hemorrhagic crusts in all patients and white yellow structure with red halo in 90.9% of patients. Pemphigus vulgaris patients were identified by the presence of dermoscopic clues such as bluish deep discoloration, tubular scaling, black dots, hair casts, hair tufts, yellow dots with whitish halos (fried egg sign) and yellow follicular pustules that are not seen in pemphigus foliaceus and IgA pemphigus. Dermoscopy is an important tool that serves as a link between clinical and histopathological diagnoses, and it can easily be used in daily practice. Several suggestive dermoscopic features can help in the differential diagnosis of autoimmune bullous disease but only after making a provisional clinical diagnosis. Dermoscopy is a very useful tool in the differentiation of pemphigus subtypes.

Line field confocal optical coherence tomography: An adjunctive tool in the diagnosis of autoimmune bullous diseases.

Autoimmune bullous diseases (AIBDs) still represent a considerable a source of morbidity and mortality: early identification of a specific AIBD is often difficult due to overlapping clinical and/or laboratory features and time-consuming invasive laboratory tests. We aimed to investigate the potential role of a new imaging technology, line-field confocal optical coherence tomography (LC-OCT), in the non-invasive diagnosis of AIBDs. LC-OCT was performed at lesional, perilesional and contralateral healthy sites in 30 patients, before histology and direct immunofluorescence. LC-OCT examination was able to identify the level of split (subcorneal/suprabasal/subepidermal/sublamina densa), to provide detailed images of the bulla roof morphology and content (eg, erythrocytes/acantholytic cells/polymorphonucleates). Areas of intra/subepidermal detachment were also detected also at clinically normal perilesional skin sites. LC-OCT can support physicians, real time and at bed-site, in the differential diagnosis of various AIBDs and their mimickers. Moreover, it can be used for the identification of subclinical lesions and therapy tapering.

Comparative Analysis of BIOCHIP Mosaic-based Indirect Immunofluorescence with Direct Immunofluorescence in Diagnosis of Autoimmune Bullous Diseases

Background:Autoimmune bullous diseases (AIBD) are a heterogeneous group of diseases characterized by autoantibodies against desmosomal proteins in the pemphigus group of disorders and adhesion molecules of the dermal-epidermal junction in pemphigoid group of diseases. Direct immunofluorescence (DIF) establishes the diagnosis of AIBD by demonstrating intercellular deposits of IgG and C3 in case of pemphigus and linear deposits of IgG and C3 along the basement membrane zone (BMZ) in bullous pemphigoid (BP). BIOCHIP mosaic-based indirect immunofluorescence (IIF), a novel diagnostic approach employs detection of characteristic staining pattern and target antigens in a single miniature incubation field.Aim:To compare the BIOCHIP mosaic-based IIF with DIF in the diagnosis of AIBD.Materials and Methods:A total of 40 patients of AIBD in the active phase of the disease were included in the study. Skin biopsy was done in these patients for DIF study and serum was subjected to BIOCHIP mosaic-based IIF assay. The results were then compared.Results:DIF revealed a diagnosis of Pemphigus in 18 patients and BP in 22 patients. BIOCHIP showed a diagnosis of pemphigus in 18 patients, BP in 18 patients and floor pattern staining in four patients, which could be attributed to any of the floor pattern staining subepidermal blistering disease.Limitations:Small sample size, lack of control group and no comparison made with ELISA.Conclusion:This study concludes that the result of BIOCHIP shows correlation with the DIF and can be used as a first line-screening tool in the diagnosis of AIBD.

Immunoglobulin and Complement Immunohistochemistry on Paraffin Sections in Autoimmune Bullous Diseases: A Systematic Review and Meta-analysis.

Immunohistochemistry (IHC) on formalin-fixed, paraffin-embedded tissue has been proposed as a potential tool in the diagnosis of autoimmune bullous diseases (AIBDs) in lieu of standard direct immunofluorescence (DIF) microscopy. To comprehensively determine the diagnostic accuracy of immunoglobulin and complement IHC for diagnosis of AIBDs, we conducted a systematic review and multivariate Bayesian model-based meta-analysis of the literature. Quality and heterogeneity assessment of studies was performed using the Quality Assessment of Diagnostic Accuracy Studies (QUADAS-2) checklist and the I2 index, respectively. Electronic searches using PubMed from April 1964 to July 2020 identified 14 articles meeting predetermined inclusion and exclusion criteria. Median sensitivities with 95% credible intervals in pemphigus and pemphigoid were 0.24 (0.01-0.89) and 0.22 (0.02-0.77) with immunoglobulin G (IgG), 0.77 (0.39-0.95) and 0.25 (0.02-0.85) with IgG4, 0.11 (0.02-0.32) and 0.86 (0.56-0.98) with C3d, and 0.84 (0.56-0.97) and 0.75 (0.37-0.94) with C4d, respectively. Specificities were 1.00 (0.00-1.00) with IgG, 0.98 (0.89-1.00) with IgG4, 0.99 (0.97-1.00) with C3d, and 0.99 (0.97-1.00) with C4d. The risk of bias and heterogeneity among studies was a serious problem, decreasing the level of evidence. Our work suggests that, in selected cases, paraffin-based IHC may be a helpful procedure to screen for AIBDs, especially when specialized laboratories and/or biopsy specimens for DIF do not exist. Nevertheless, more studies with a refined quality design are needed to explore the true usefulness of this diagnostic method in AIBDs.

BIOCHIP mosaic for the diagnosis of autoimmune bullous diseases in Chinese patients.

Autoimmune blistering diseases (AIBDs) are a group of fatal diseases with specific autoantibodies. BIOCHIP mosaic is a novel and all-in-one measure used for the rapid diagnosis of AIBDs. To evaluate the diagnostic accuracy based on BIOCHIP mosaic (FA1501-1005-60) in Chinese patients with AIBDs. Seventy-seven patients with AIBDs and 20 controls were enrolled. The BIOCHIP mosaic was performed using both serum and plasma samples. Based on BIOCHIP mosaic, the data from paired plasma and serum samples demonstrated a high degree of concordance (Cohen's kappa = 0.896-1.000) for autoantibodies against Dsg1, Dsg3, BP180-NC16A-4X, BP230gC, prickle-cell desmosomes, and pemphigoid antigens. Moreover, BIOCHIP mosaic also demonstrated a high degree of consistency for the detection rate of anti-Dsg1, Dsg3, plakins, BP180-NC16A-4X and non-collagenous domain of type VII collagen autoantibodies for the diagnosis of pemphigus foliaceus (77.3%), pemphigus vulgaris (88.6%), paraneoplastic pemphigus (100.0%), bullous pemphigoid (92.8%) and epidermolysis bullosa acquisita (99.0%), respectively. Using BIOCHIP mosaic, serum and plasma samples may be used interchangeably at 1/10 dilution. Overall, the BIOCHIP mosaic was shown to be a useful and accurate tool for the diagnosis of AIBDs.

Detection of circulating anti-skin antibodies by indirect immunofluorescence and by ELISA: a comparative systematic review and meta-analysis.

Background Both enzyme-linked immunosorbent assays (ELISAs) and indirect immunofluorescence (IIF) are available for the diagnosis of autoimmune bullous diseases (AIBD). Many studies have reported on the performance of ELISAs and concluded that ELISAs could replace IIF. This study compares the diagnostic accuracy of ELISA and IIF for the detection of autoantibodies to desmoglein 1 (DSG1), desmoglein 3 (DSG3), bullous pemphigoid antigen 2 (BP180) and bullous pemphigoid antigen 1 (BP230) to support the diagnosis of pemphigus vulgaris (PV), pemphigus foliaceus (PF) and bullous pemphigoid (BP). Methods A literature search was performed in the PubMed database. The meta-analysis was performed using summary values and a bivariate random effect model. Results The five included studies on PV did not demonstrate significant differences between IIF and DSG3-ELISA (sensitivity 82.3% vs. 81.6%, p = 0.9284; specificity 95.6% vs. 93.9%, p = 0.5318; diagnostic odds ratio [DOR] 101.60 vs. 67.760, p = 0.6206). The three included studies on PF did not demonstrate significant differences between IIF and DSG1-ELISA (sensitivity 80.6% vs. 83.1%, p = 0.8501; specificity 97.5% vs. 93.9%, p = 0.3614; DOR 160.72 vs. 75.615, p = 0.5381). The eight included studies on BP showed that BP230-ELISA differed significantly from both IIF on monkey esophagus (MO) and BP180-ELISA with regard to DOR (11.384 vs. 68.349, p = 0.0008; 11.384 vs. 41.699, p = 0.0125, respectively) Conclusions Our meta-analysis shows that ELISA performs as well as IIF for diagnosing PV, PF and BP.

Multicenter prospective study on multivariant diagnostics of autoimmune bullous dermatoses using the BIOCHIP technology

Thecurrent 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. 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 HEK293cells 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 Lübeck. 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. Laminin 332 and laminin γ1 are not represented on the BIOCHIP Mosaic. The BIOCHIP Mosaic is a standardized time- and serum-saving approach that further facilitates the serologic diagnosis of AIBD.

A Monocentric Retrospective Observational Study of Comorbidities in Patients Affected by Autoimmune Bullous Diseases.

Autoimmune bullous diseases (AIBDs) of the skin and mucosae include a heterogeneous group of chronic diseases, which could be associated with various comorbidities. The purpose of this study was to evaluate the comorbidity profiles of patients affected by AIBDs, who referred to the Dermatological Clinic of Padua from December 2015 to June 2018. A monocentric retrospective observational study was conducted on 157 patients with diagnosis of AIBDs. Patients' comorbidities were investigated during the periodic visits of follow-up and through the analysis of computerized medical records. Among the 157 patients, 40 (25.5%) were diagnosed with PV, 15 (9.6%) with PF, and 102 (64.9%) with BP. Nine different comorbidities were observed, but only two of these were statistically significantly associated with BP: type 2 diabetes (p=0.0142) and neuropsychiatric disorders (p=0.015). BP is statistically significantly associated with type 2 diabetes mellitus and neuropsychiatric diseases. The correlation with neuropsychiatric pathologies is interesting for the possible bidirectional role in their etiology. The association with type 2 diabetes mellitus could suggest more caution in the administration of systemic corticosteroids, especially in elderly patients.

Comparative Analysis of BIOCHIP Mosaic-Based Indirect Immunofluorescence with Direct Immunofluorescence in Diagnosis of Autoimmune Bullous Diseases: A Cross-Sectional Study.

Background:Autoimmune bullous diseases (AIBD) are a heterogeneous group of diseases characterized by autoantibodies against desmosomal proteins in the pemphigus group of disorders and adhesion molecules of the dermal-epidermal junction in pemphigoid group of diseases. Direct immunofluorescence (DIF) establishes the diagnosis of AIBD by demonstrating intercellular deposits of IgG and C3 in case of pemphigus and linear deposits of IgG and C3 along the basement membrane zone (BMZ) in bullous pemphigoid (BP). BIOCHIP mosaic-based indirect immunofluorescence (IIF), a novel diagnostic approach employs detection of characteristic staining pattern and target antigens in a single miniature incubation field.Aim:To compare the BIOCHIP mosaic-based IIF with DIF in the diagnosis of AIBD.Materials and Methods:A total of 40 patients of AIBD in the active phase of the disease were included in the study. Skin biopsy was done in these patients for DIF study and serum was subjected to BIOCHIP mosaic-based IIF assay. The results were then compared.Results:DIF revealed a diagnosis of Pemphigus in 18 patients and BP in 22 patients. BIOCHIP showed a diagnosis of pemphigus in 18 patients, BP in 18 patients and floor pattern staining in four patients, which could be attributed to any of the floor pattern staining subepidermal blistering disease.Limitations:Small sample size, lack of control group and no comparison made with ELISA.Conclusion:This study concludes that the result of BIOCHIP showed a significant correlation with the DIF and can be used as a first line-screening tool in the diagnosis of AIBD.

Trichoscopy – a useful tool in the preliminary differential diagnosis of autoimmune bullous diseases

Scalp is a common location of autoimmune bullous diseases. Trichoscopy is a noninvasive method for diagnosing hair and scalp diseases. Data on trichoscopy in autoimmune bullous diseases are limited to the studies on pemphigus including a small number of patients. Trichoscopic characteristics of bullous pemphigoid and dermatitis herpetiformis were not reported to date. The aim of the study was to determine the value of trichoscopy in the differential diagnosis of pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid, and dermatitis herpetiformis. Trichoscopy was used to evaluate scalp lesions in 68 patients (26 with pemphigus vulgaris, 17 with pemphigus foliaceus, 17 with bullous pemphigoid, and 8 with dermatitis herpetiformis). The working magnification was 20-fold and 70-fold. The most frequent trichoscopic feature of autoimmune bullous diseases was extravasations. They occurred in 76.9% of patients with pemphigus vulgaris, 70.6% of patients with pemphigus foliaceus, 76.5% of patients with bullous pemphigoid, and 100% of patients with dermatitis herpetiformis. Yellow hemorrhagic crusts occurred in, respectively, 73.1%, 70.6%, 64.7%, and 35.5% of the cases. Yellow diffuse scaling and tubular scaling occurred more frequently in pemphigus foliaceus (52.9% and 41.2%, respectively). Clustered dotted vessels were characteristic for dermatitis herpetiformis (5/8, 62.5%). Dotted vessels with whitish halo were a hallmark of pemphigus vulgaris. A trichoscopic algorithm for the differential diagnosis of autoimmune bullous diseases was developed. Autoimmune bullous diseases present characteristic trichoscopic patterns. Trichoscopy can be regarded as a rapid in-office preliminary diagnostic method in the differential diagnosis of these diseases.

C4d and C3d immunohistochemical evaluation on formalin-fixed paraffin-embedded tissue for the diagnosis of bullous pemphigoid: systematic review of the literatures

Introduction: Several methods are available for the diagnosis of autoimmune bullous disease. Since the immunohistochemistry of complement component is easy and more accessible compared to other methods, it is thought that this technique as an efficient method can replace other difficult, and time-consuming procedures. Therefore, in this study we aimed to systematically review the literatures in which the diagnostic value of complement component 3d (C3d) and C4d had been investigated in bullous pemphigoid. Methods: A systematic search was conducted in the PubMed, Google scholar, and Scopus using following search method (((C3d OR C4d OR complement component 3d OR complement component 4d immunohistochemistry)) OR (C3d OR C4d marker OR complement component 3d OR complement component 4d marker)) AND (bullous pemphigoid OR cutaneous pemphigoid) to evaluate the diagnostic value of C3d and/or C4d for early and accurate detection of bullous pemphigoid on November 2015. Subsequently, the extracted data were described.Result: Total of 28 documents were collected and reviewed based on the purpose of this study. Of the collected articles, 21 documents were excluded in several steps of article selection process and only 7 relevant articles were included for data assessment. The results showed that the deposits of C3d and/or C4d in skin biopsies were found in 125 of 134 patients, indicating that immunohistochemistry is a reliable technique for the diagnosis of inflammatory skin diseases.Conclusion: The results of this review showed that C3d and/or C4d immunohistochemistry in skin biopsies is a reliable technique for the diagnosis of inflammatory skin diseases, particularly bullous pemphigoid.

Prospective studies on the routine use of a novel multivariant enzyme-linked immunosorbent assay for the diagnosis of autoimmune bullous diseases

Serologic diagnosis of autoimmune blistering disease (AIBD) usually follows a sophisticated multistep algorithm. We sought validation of a multivariant enzyme-linked immunosorbent assay (ELISA) in the routine diagnosis of AIBD. The multivariant ELISA comprising 6 recombinant immunodominant forms of major AIBD target antigens, ie, desmoglein 1, desmoglein 3, envoplakin, BP180, BP230, andtype VII collagen was applied in: (1) a cohort of well-characterized AIBD (n=173) and control sera (n=130), (2) a prospective multicenter study with 204 sera from patients with newly diagnosed AIBD with positive direct immunofluorescence microscopy, and (3) a prospective monocenter study with 292 consecutive sera from patients with clinical suspicion of AIBD in comparison with the conventional multistep diagnostic algorithm. Concordant results in the multivariant ELISA compared with direct immunofluorescence microscopy were seen in 94% of patients with pemphigus and 71% of patients with pemphigoid (Cohen κ value, 0.95 and 0.66) and with the conventional multistep diagnostic approach in 91% of patients with pemphigus and 88% of patients with bullous pemphigoid and 93% of autoantibody-negative sera (Cohen κ, 0.95, 0.84, and 0.78). IgA autoantibodies and less common target antigens were not analyzed. The multivariant ELISA is a practical, highly standardized, and widely available novel diagnostic tool for the routine diagnosis of AIBD.

The Role of Anti-skin Antibodies Immunoglobulin G in the Diagnosis of Autoimmune Bullous Diseases

The Role of Anti-skin Antibodies Immunoglobulin G in the Diagnosis of Autoimmune Bullous Diseases

Serological Diagnosis of Autoimmune Blistering Diseases

Autoimmune blistering diseases are a rare diseases, characterized by development of autoantibodies against the structural proteins of the epidermis or dermoepidermal junction, and blisters and erosions on skin and/or mucous membranes clinically. Clinical features are important guiding findings for suspicious of this group of diseases. The diagnosis is achieved by the evaluation together of clinical features, histological and immunological findings. The gold standard in the diagnosis of this group diseases are demonstration of tissue bound and/or circulating autoantibodies. Methods for this purpose are; direct and indirect immunofluorescence, Enzyme Linked Immunosorbent Assay (ELISA), immunoprecipitation and immunoblotting. The aim of this paper is to review serological diagnostic methods in the diagnosis of autoimmune bullous diseases and to present developments in recent years.

Diagnosis of autoimmune bullous disease in oral cavity observed as refractory stomatitis

Diagnosis of autoimmune bullous disease in oral cavity observed as refractory stomatitis

A cross-sectional study of clinical, histopathological and direct immmunofluorescence diagnosis in autoimmune bullous diseases

Background: Immunobullous diseases are morphologically heterogeneous and the differentiation between various subtypes is essential for proper treatment and prognosis. Aim of our study was to analyze and correlate clinical, histopathological and immunofluorescence findings in autoimmune bullous diseases. Materials and Methods: A cross-sectional study was done over a period of two years (2010-2012) after approval of the ethics committee. Sixty patients, who met the inclusion criteria of immunobullous disease, were included in the study. Skin biopsy for histopathology and direct immunofluorescence (DIF) examination was taken. DIF using salt-split technique was done in few of the cases. The final diagnosis was based on clinical, histopathology and DIF findings. Pearson's coefficient of correlation (r) was calculated. Statistical Analysis was done using Epi info version. 7.0. Results: Fifty-three cases with clinical diagnosis of autoimmune bullous diseases were evaluated. In 88.6% of cases, histopathology diagnosis was consistent with clinical diagnosis and in 75.5% of cases, DIF findings were consistent with clinical diagnosis. A positive relation was seen between clinical and DIF findings with r = 0.65 and between histopathology and DIF findings with r = 0.75. DIF positivity was seen in 100% cases of bullous pemphigoid (BP) and pemphigus foliaceous and 94.7% cases of pemphigus vulgaris, which was statistically significant with p < 0.05. In DIF salt-split test, deposition was seen on roof of blister in BP whereas on floor in epidermolysis bullosa acquisita. Conclusion: Our study provides evidence-based guidance for the diagnosis and classification of various immunobullous disorders. DIF test should be done in conjunction with histopathology for definitive diagnosis and to minimize both: False-positive and false-negative results.

Infection in autoimmune bullous diseases: A retrospective comparative study

Certain autoimmune bullous diseases (AIBD), including pemphigoid and pemphigus, confer increased infection risk. Infections have not been systematically studied in these conditions, however. Little is known about infection risk in these conditions, particularly dermatitis herpetiformis (DH). We aimed to characterize and compare infection patterns and risk factors in patients with pemphigoid, pemphigus, and DH. We retrospectively studied the medical records of Olmsted County, Minnesota, residents who had a diagnosis of AIBD between 1January 1998 and 1January 2011. Of 81 patients studied, 54 (67%) had pemphigoid, 11 (14%) had pemphigus and 16 (20%) had DH. Most patients studied developed at least one localized infection (72%) or one systemic infection (83%). Almost one-third of patients (31%) developed infections requiring hospitalization or contributing to death. All patients taking systemic corticosteroids experienced a localized or systemic infection during the follow-up period. Systemic infections were significantly less frequent in patients with DH than those with pemphigoid or pemphigus (P=0.03), as were systemic infections requiring hospitalization or contributing to death (P=0.002). Patients with DH were significantly less likely to require systemic corticosteroids (P<0.001) and significantly more likely to receive dapsone (P=0.002). The study design was retrospective and a limited number of patients met the inclusion criteria. Patients with AIBD frequently developed localized and systemic infections, a substantial portion of which contributed to hospitalization or death. Patients with DH experienced infections of lesser severity than patients with pemphigoid or pemphigus.

Autoimmune Blistering Diseases: Incidence and Treatment in Croatia

Dermatovenereology in Croatia has a long tradition, with the first Department of Dermatovenereology having opened in Zagreb in 1894. The diagnosis of autoimmune bullous disease is based on clinical, histopathologic, and immunopathologic findings. Future goals are to participate in multicenter studies because this is the only possibility for further investigation for these rare diseases.