Bullous Pemphigoid Disease Area Index Research Articles

15014 Cross-sectional study of the prevalence and predictors of pruritus in pemphigus as compared with bullous pemphigoid

Pemphigus is an autoimmune blistering disease that causes painful erosions, while bullous pemphigoid (BP) is classically known to cause pruritic blisters. Previous reports and anecdotal clinical observations suggest pemphigus patients also experience significant itch; we explored the prevalence and potential predictors of itch in pemphigus patients compared with BP. While the Bullous Pemphigoid Disease Area Index (BPDAI) has a subjective patient itch score (0-10 over 24 hours, week, and month), the Pemphigus Disease Area Index (PDAI) does not, so the BPDAI Pruritus component was administered to both BP and pemphigus patients.

More Severe Erosive Phenotype Despite Lower Circulating Autoantibody Levels in Dipeptidyl Peptidase-4 Inhibitor (DPP4i)-Associated Bullous Pemphigoid: A Retrospective Cohort Study.

BackgroundThe clinical and immunological profile of patients with dipeptidyl peptidase-4 inhibitor (DPP4i)-associated bullous pemphigoid (BP) is inconsistent in the current literature.ObjectivesThe aims were to investigate the clinical and immunological features of patients with DPP4i-associated BP and to examine whether there are intraclass differences between different DPP4i agents.MethodsA retrospective cohort study was conducted, including all consecutive patients diagnosed with BP throughout the years 2009–2019 in a tertiary referral center.ResultsThe study encompassed 273 patients with BP (mean age at diagnosis 79.1 ± 9.9 years), of whom 24 (8.8%) were associated with DPP4i. Sitagliptin was the prescribed agent for 17 patients (70.8%), and vildagliptin was prescribed in seven patients (29.2%). Relative to other patients with BP, patients with DPP4i-associated BP had more prominent truncal involvement (95.8% vs. 73.9%; P = 0.017), greater erosion/blister Bullous Pemphigoid Disease Area Index (BPDAI) subscore (29.8 ± 17.4 vs. 20.6 ± 14.4; P = 0.018), and lower levels of anti-BP180 NC16A (279.2 ± 346.1 vs. 572.2 ± 1352.0 U/ml; P = 0.009) and anti-BP230 (25.5 ± 47.8 vs. 128.6 ± 302.9 U/ml; P = 0.009) antibodies. Relative to patients with sitagliptin-associated BP, those with vildagliptin-associated BP had a lower seropositivity rate (57.1% vs. 94.1%, P = 0.031) and lower levels (96.7 ± 139.0 vs. 354.5 ± 376.5; P = 0.023) of anti-BP180 NC16A antibodies, and tended to present with higher erosion/blister BPDAI subscore (36.3 ± 9.6 vs. 25.8 ± 19.7; P = 0.095).ConclusionsDPP4i-associated BP is characterized by a more severe blistering and erosive presentation despite lower levels of typically pathogenic antibodies.

Total IgE, eosinophils, and interleukins 16, 17A, and 23 correlations in severe bullous pemphigoid and treatment implications.

Bullous pemphigoid (BP) patients are predominantly above 70 years of age, with limited tolerance to the side effects of the immunosuppressive drugs. Advancements in our understanding of the pathophysiology of BP have led to the development of molecules which target specific pathways involved in induction and perpetuation of disease. Patients with BP Disease Area Index above 60 and less than 100 were split into two groups-one with high and the other with normal levels of IgE. The tested parameters included eosinophils' count, total IgE serum level, and interleukins (IL) 16, 17A, and 23 counts in the peripheral blood and skin bulla serum, before any therapeutic intervention. Thirty individuals fulfilled the criteria for enrollment. Patients with high IgE blood serum levels had significantly higher levels of IL17A and normal IL23 levels in blood and bulla serum. Patients with normal serum IgE levels had slightly higher IL23 levels in blood and bulla serum. The eosinophil count was positively related to IL17 blood serum level and negatively related to IL23. IL16 did not differ in the two groups. BP patients may represent a group of patients benefiting most substantially from the introduction of nonimmunosuppressive therapeutics into the treatment regimens for their disease. Clinical criteria and immune biomarkers are needed for making the best therapeutic choice.

Itch-related quality of life impact across 3 autoimmune blistering diseases: a retrospective cohort study

Introduction: Autoimmune blistering diseases (AIBD) produce a range of debilitating symptoms potentially greatly impacting quality of life (QoL). We sought to compare the disease-related and itch-related QoL impact between AIBD including pemphigus, bullous pemphigoid (BP), and mucous membrane pemphigoid (MMP). Methods: We performed a retrospective cohort study of patients attending a specialty Autoimmune Blistering Disease Clinic at a single academic center between July 2017 and April 2019. Patients with an established histopathologic or serological diagnosis of pemphigus, BP or MMP with mucosal and/or cutaneous lesions on clinical examination, or patient-reported QoL impact in the past week were recruited to participate. Patients completed Autoimmune Blistering Disease Quality of Life (ABQoL), Treatment of Autoimmune Blistering Disease Quality of Life (TABQoL), and ItchyQoL survey instruments, and disease severity was assessed using the Pemphigus Disease Area Index (PDAI) (pemphigus), Bullous Pemphigoid Disease Area Index (BPDAI) (BP), and Mucous Membrane Pemphigoid Disease Area Index (MMPDAI) (MMP). Statistical analyses were performed using IBM SPSS 26. Results: BP and pemphigus demonstrated similar itch-related QoL impact, greater than that of MMP. Disease severity was significantly associated with itch-related QoL in BP and pemphigus, as was use of biological/targeted therapies in BP alone. Discussion: Itch is a significant symptom in both BP and pemphigus and can negatively affect QoL. Disease severity and treatment type play a role in itch-related QoL in pemphigus and BP. Additional studies are needed to elucidate the mechanisms for itch in AIBD, particularly in pemphigus where this symptom has only recently been described.

Doxycycline as an initial treatment of bullous pemphigoid in Japanese patients

AbstractObjectivesTetracycline antibiotics have been used in bullous pemphigoid (BP) for their anti‐inflammatory effects. In Japan, tetracycline and minocycline, but not doxycycline, have been generally used in BP, although doxycycline has a better side‐effect profile than other tetracycline antibiotics. To determine the effectiveness and safety of doxycycline in Japanese BP patients, we performed a retrospective cohort study.MethodsWe analyzed 27 Japanese BP patients in whom doxycycline was used as a first‐line treatment at Kido Hospital between April 1, 2014, and August 31, 2019. We estimated time to prednisolone treatment as a primary measure of effectiveness of doxycycline, because second‐line prednisolone treatment was initiated when each physician judged that disease activity could not be controlled with doxycycline alone.ResultsDuring the median follow‐up of 63 weeks, second‐line prednisolone treatment was initiated in 22 of 27 patients. Median time to prednisolone treatment was 7 weeks. Severe disease based on the Bullous Pemphigoid Disease Area Index (BPDAI) and eosinophilia were the risk factors for prednisolone treatment. At 6 weeks, 14 of 27 patients were under doxycycline treatment without prednisolone, and significant decreases of BPDAI scores were achieved. In 9 of these 14 patients, prednisolone treatment was started after 6 weeks, but the recurrent or mild remaining lesions could be controlled with relatively low‐dose prednisolone in combination with doxycycline. No severe adverse events related to doxycycline occurred.ConclusionsDoxycycline has short‐term effectiveness, potential corticosteroid‐sparing effect, and long‐term safety in Japanese BP patients with mild to moderate disease and no eosinophilia.

Clinical characteristics of pruritus in patients with bullous pemphigoid: a preliminary questionnaire-based study.

IntroductionBullous pemphigoid (BP) is the most common autoimmune blistering disease. Although pruritus is a leading symptom in BP, its specific characteristics have not been explored.AimTo determine the itch characteristics in newly diagnosed BP patients by using the Questionnaire of Descriptive Assessment of Pruritus and visual analogue scale (VAS) and to correlate itch with disease severity.Material and methodsThe study included 32 patients with BP (mean age: 75.4 ±12.2 years; 20 women, 12 men), who filled in the questionnaire. Bullous Pemphigoid Disease Area Index (BPDAI) was assessed.ResultsIn 78.1% of BP patients pruritus occurred at least once daily. Pruritus was most frequent in the evening and at night and frequently persisted for more than 10 min in more than a half of patients (long itch episodes). Aggravating factors were sweating (50.0%), heat (59.4%) and stress (46.9%), but the major relieving factor was cold (34.4%). Itch intensity in BP was assessed as moderate (mean VAS score: 5.8 points) and did not correlate with disease severity (BPDAI). BP patients declared difficulties caused by pruritus: falling asleep (53.1%) and awakening at night (50%).ConclusionsThis study provided detailed characteristics in BP patients confirming that pruritus is classified as an important troublesome symptom. Therefore there is a need to search for therapeutic solutions.

Basement membrane zone IgE deposition is associated with bullous pemphigoid disease severity and treatment results.

A subset of patients with bullous pemphigoid (BP) show deposition of IgE in the basement membrane zone (BMZ), yet the relationship between BMZ IgE and the clinical presentation of BP remains unclear. To investigate the relationship between IgE deposition, IgE levels in serum, and disease severity in patients with BP. We investigated IgE autoantibodies in 53 patients with BP by direct immunofluorescence (DIF), indirect immunofluorescence and enzyme-linked immunosorbent assay. Of 53 patients with BP, 23 (43%) had IgE deposition, 10 (19%) of whom were IgE+ and 13 (25%) IgE± according to DIF analyses. Erosion/blister (E/B) Bullous Pemphigoid Disease Area Index (BPDAI) scores were significantly higher in IgE+ patients than in IgE- patients (n = 15), while no significant differences were found for urticaria/erythema BPDAI scores. IgE+ and IgE± patients took longer to reduce their E/B BPDAI score by 75% after systemic corticosteroid treatment. BP180-IgE levels were significantly higher among IgE+ patients than IgE± or IgE- patients (n = 10). Total IgE levels in the serum and blood eosinophil counts did not differ between IgE+, IgE± and IgE- patients. A significant correlation was detected between BP180-IgG and BP180-IgE, but not between BPDAI scores and any of BP180-IgG, BP180-IgE or blood eosinophil count. IgE deposition in the BMZ is associated with higher E/B BPDAI scores and longer treatment periods. We conclude that IgE binding in the BMZ may contribute to BP pathogenesis by promoting blister formation. What's already known about this topic? BP180-IgE autoantibodies have an important role in the pathogenesis of bullous pemphigoid (BP). A subset of patients with BP display deposition of IgE within the basement membrane zone (BMZ) of skin tissue. What does this study add? Patients with invivo IgE deposition in the BMZ displayed higher erosion/blister Bullous Pemphigoid Disease Area Index (BPDAI) scores, while urticaria/erythema BPDAI scores were not significantly different. Patients with invivo IgE deposition in the BMZ took longer to reduce their erosion/blister BPDAI score by 75% after systemic corticosteroid treatment. BP180-specific IgE levels in serum were higher among patients with linear IgE deposition in the BMZ than in those with granular or no IgE deposition.

025 Analysis of the related factors that leading to the resistance of topical treatment for bullous pemphigoid patients

Based on clinical and laboratory data, we analyzed the related factors leading to the resistance of topical treatment for BP patients. Totally 64 BP patients were enrolled in the study, and divided into two groups according to different methods of treatment. One of the groups contained 22 patients that showed effective to topical treatment. Another group contained 42 patients, who got more than 3 new blisters in the continuous 3 days during 4 weeks of the topical treatment, which showed resistant to topical treatment. The types of lesions, the BPDAI (Bullous Pemphigoid Disease Area Index score, Bullous Pemphigoid Diseases Area Index) , the concentration of albumin, eosinophil counts, the titer of anti-BP180 and anti-BP230 IgG, the concentration of total IgE, the titer of anti-BP180 and anti- BP230 IgE of the two groups were analyzed by GraphPad Prism5 software, and Mann Whitney inspection methods were used for statistical analysis. The results of analysis will be helped to select the proper treatments for different BP patients. The results showed that the main lesion type of the group that showed effective to topical treatment was simple blister (68 %), and the type of erythema with blister (63%) was the major type of the patients that resistant to topical treatment. The BPDAI, EOS counts, the concentration of total IgE, the titer of anti-BP180 IgG, anti-BP230 IgG, and anti-BP230 IgE were significantly higher in the group of patients that effective to topical treatment (P < 0.05). There’s no difference of the concentration of albumin and the titer of anti-BP180 IgE between the two groups. So according to our study, in addition to BPDAI and anti-BP180 IgG titer, we can also select appropriate treatments for BP patients according to their lesion type, peripheral eosinophil counts, the concentration of total IgE, and the titer of anti-BP230 IgE.

Actigraphy-measured nocturnal wrist movements and assessment of sleep quality in patients with bullous pemphigoid: a pilot case-control study.

Bullous pemphigoid (BP) is a distressing autoimmune bullous disease strongly associated with severe pruritus; however, data concerning pruritus in BP are still scarce. No clinical research evaluating the effect of BP on sleep quality has been conducted. To evaluate the intensity of pruritus measured by nocturnal wrist movements (NWMs) and the sleep quality in patients with BP using actigraphy in comparison with nonpruritic healthy controls (HCs) with subsequent correlations with an itch visual analogue scale (VAS) as a subjective measure, disease severity [Bullous Pemphigoid Disease Area Index (BPDAI), urticaria/erythema, erosions/blisters] and serum total IgE level. In total, 31 patients with newly diagnosed BP (mean±SD age 75.4±12.3years) and 40 nonpruritic HCs (age 73.5±11.7years) were recruited. All participants wore a sleep monitor (ActiSleep+) on the dominant wrist. For patients with BP, median VAS score was 5.5 and median BPDAI was 43 (urticaria/erythema BPDAI was 16, erosions/blisters BPDAI was 29). Scratching, defined as bouts of NWMs, was significantly (P<0.001) more intensive in patients with BP than in controls. Characteristic of BP was that scratching bouts corresponded with the slowest wrist movements. There were no correlations with VAS, BPDAI or total IgE level. Compared with HCs, patients with BP presented significant (P<0.001) sleep disturbances, as determined by sleep efficiency, waking after sleep onset and average duration of awakening, and these were strongly correlated with urticaria/erythema BPDAI. Nocturnal wrist movements measured by actigraphy are more intensive in patients with BP than in nonpruritic HCs, and characteristically slow movements. Actigraphy method showed very low sleep quality in patients with BP, thus severity of BP has a negative impact on sleep.

There is no correlation between the serum level of interleukin-21 and bullous pemphigoid

Objective To investigate the correlations of the serum level of interleukin-21 (IL-21) with anti-BP180 antibodies, peripheral eosinophil level, bullous pemphigoid disease area index (BPDAI) in patients with bullous pemphigoid (BP) . Methods A total of 31 patients with bullous pemphigoid and 31 healthy controls were enrolled into this study. Blood samples were collected from all the subjects, and enzyme-linked immunosorbent assay (ELISA) was performed to detect the serum level of IL-21 and serum titer of anti-BP180 antibodies. BPDAI was assessed among 13 patients, and the correlation of the IL-21 level with anti-BP180 antibody titers, eosinophil count and BPDAI were analyzed. Results No significant difference in the serum level of IL-21 was observed between the patients (M[P25 - P75]: 255.28 [156.19 - 361.59] ng/L) and healthy controls (150.47 [72.4 - 268.73] ng/L, P = 0.14) . There was no correlation between the serum level of IL-21 and serum titer of anti-BP180 antibodies (82.98 [26.82 - 132.92]U/ml, rs = 0.18, P = 0.332) . Among the 27 BP patients who underwent blood cell count, no correlation was found between the serum levels of IL-21 and whole-blood eosinophil counts (rs = 0.104, P = 0.606) . Among the 13 BP patients who underwent assessment of BPDAI, there was no correlation between the serum levels of IL-21 and BPDAI scores (rs = 0.233, P = 0.464) . Conclusion The IL-21 level is uncorrelated with anti-BP180 antibody level, peripheral eosinophil level or BPDAI in patients with BP. Key words: Pemphigoid, bullous; Eosinophils; Interleukin-21; Anti-BP180 antibody

BPDAI and ABSIS correlate with serum anti-BP180 NC16A IgG but not with anti-BP230 IgG in patients with bullous pemphigoid.

Bullous pemphigoid (BP) is an autoimmune bullous disease characterized by autoantibody production against BP180 and BP230. Two scoring systems have been validated for BP including: Bullous Pemphigoid Disease Area Index (BPDAI) and Autoimmune Bullous Skin Disorder Intensity Score (ABSIS). In this study, we investigated correlations between both scoring systems and either anti-BP180 NC16A or anti-BP230 values. BPDAI and ABSIS were used to measure disease activity in 95 BP patients at Razi Hospital in Tehran, Iran. ELISA was performed on patient sera to identify any significant relationship between anti-BP180 NC16A/anti-BP230 values and BP disease activity. The two scores showed a strong correlation (ρ = 0.73; p value < 0.0001). Anti-BP180 NC16A values correlated with BPDAI (ρ = 0.49, p value = 0.0001), ABSIS (ρ = 0.47, p value < 0.0001), and BPDAI-Pruritus scores (ρ = 0.29, p value < 0.005). There was a strong correlation between anti-BP180 NC16A values and the ABSIS Skin score (ρ = 0.58, p value < 0.0001), and a moderate correlation with erosion/blister BPDAI score (ρ = 0.48, p value < 0.001) and urticaria/erythema BPDAI score (ρ = 0.27, p value = 0.009). Anti-BP230 values did not demonstrate any relationship with either scores or their subcomponents. Both scoring systems demonstrated moderate validity. Mucosal components did not show any correlation with anti-BP antibodies and are disproportionately presented in both BP scoring tools.

HLA-DQB1*03:01 as a Biomarker for Genetic Susceptibility to Bullous Pemphigoid Induced by DPP-4 Inhibitors

HLA-DQB1*03:01 as a Biomarker for Genetic Susceptibility to Bullous Pemphigoid Induced by DPP-4 Inhibitors

The Reliability, Validity and Responsiveness of Two Disease Scores (BPDAI and ABSIS) for Bullous Pemphigoid: Which One to Use?

A significant obstacle in guiding evidence-based management of bullous pemphigoid (BP) is the lack of a standardised, validated scoring system for the condition. The aim of this study is to evaluate the suitability of the Bullous Pemphigoid Disease Area Index (BPDAI) and the Autoimmune Bullous Skin disorder Intensity Score (ABSIS) as outcome measures for BP in clinical trials. Thirty-two BP patients were repeatedly assessed over four years using Physician Global Assessment (PGA), anti-BP180 ELISA titres, BPDAI, ABSIS, BPDAI-Pruritus, Autoimmune Bullous Disease Quality of Life (ABQOL) and Treatment of Autoimmune Bullous Disease Quality of Life (TABQOL) questionnaires. The reliability, validity, responsiveness, and minimal clinically important differences (MCIDs) were calculated. For inter-rater reliability, the intraclass correlation coefficients (95% CI) were: BPDAI 0.957 (0.901-0.982) and ABSIS 0.881 (0.736-0.949). Compared to ABSIS, BPDAI was better correlated with PGA(r = 0.875, p < 0.001), BPDAI-Pruritus (r=0.632, p = 0.004), ABQOL (r = 0.521, p = 0.011) and TABQOL (r=0.538, p = 0.008). MCIDs for BPDAI were 4-points for assessing clinical improvement and 3-points for deterioration. ABSIS demonstrated less responsiveness with MCIDs at 8.6-points for improvement and 4-points for deterioration. These results indicate that BPDAI demonstrated excellent reliability, validity and responsiveness, while ABSIS had moderate to good reliability, validity and responsiveness.

Validation of chemiluminescent enzyme immunoassay in detection of autoantibodies in pemphigus and pemphigoid

BackgroundA novel chemiluminescent enzyme immunoassay (CLEIA) was recently developed to quantify autoantibodies specific for desmogleins (Dsgs) and BP180, the target antigens of pemphigus and pemphigoid. This assay is automated and highly accurate and efficient. ObjectiveTo validate the use of the CLEIA for detection of autoantibodies during the clinical courses of patients with pemphigus and pemphigoid. MethodsTo define cut-off values for Dsg1, Dsg3, and BP180, we evaluated 47 serum samples from patients with pemphigus foliaceus (PF), 59 from those with pemphigus vulgaris (PV), 52 from those with bullous pemphigoid (BP), and 995 from healthy individuals. We also evaluated any fluctuations in CLEIA titers according to disease activity during the clinical course of 10 cases each of PF, PV, and BP. We used clinical symptom scores, the pemphigus disease area index (PDAI) and the bullous pemphigoid disease area index (BPDAI), to evaluate disease activity. ResultsThe cut-off values for the CLEIA titers determined by the Youden index were 15.4U/mL for Dsg1, 14.9U/mL for Dsg3, and 16.8U/mL for BP180. CLEIA titers fluctuated in parallel with the PDAI/BPDAI scores in 28 of the 30 cases with PF, PV, or BP. Although the CLEIA and enzyme-linked immunosorbent assay (ELISA) values in the same samples differed substantially in some cases, the concordance rates of positive/negative results between the CLEIA and ELISA were 96% for Dsg1, 97% for Dsg3, and 96% for BP180. ConclusionThe CLEIA, a newly developed, highly effective autoantibody detection system, is as reliable as ELISA for evaluation of the clinical courses of pemphigus and pemphigoid.

Clinical measures of bullous pemphigoid

Bullous pemphigoid (BP) is a group of autoimmune blistering skin diseases. There are special measures for the assessment of disease severity and therapeutic response in autoimmune bullous skin diseases. Common scoring indexes include autoimmune bullous skin disorder intensity score (ABSIS), bullous pemphigoid disease area index (BPDAI), and so on. ABSIS has been validated, and is widely applied in evaluation of pemphigus vulgaris and other autoimmune blistering diseases. BPDAI is still in the process of verification, and has been applied in only a few clinical trials. The evaluation of diseases by ABSIS and BPDAI is both based on a global assessment of skin and mucosal lesions, and degree of subjective pruritus. Key words: Pemphigoid, bullous; Autoimmunity; Severity of illness score; Bullous pemphigoid disease area index; Clinical outcome measures

Outcome measures for autoimmune blistering diseases.

Outcome measures are crucial in assessing an autoimmune blistering disease's (AIBD) severity as well as its impact on the patient's quality of life (QOL). The standardization of AIBD outcome measures is pivotal to accurately monitor the patient and to pool results from randomized controlled trials for meta-analysis, and thereby provide knowledge of the optimal AIBD therapies. In the past decade, several AIBD severity outcome measures have been developed and validated. For pemphigus severity, the Pemphigus Disease Area Index (PDAI) developed by the International Pemphigus Definitions Group was shown to be the most superior, followed by the Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) by the German group. For bullous pemphigoid severity, the Bullous Pemphigoid Disease Area Index (BPDAI) was shown to be an accurate and valid measure. To quantify the burden of AIBD and its treatments on QOL, the Autoimmune Bullous Disease Quality of Life (ABQOL) and the Treatment of Autoimmune Bullous Disease Quality of Life (TABQOL) were also developed, validated, and are now being validated in multiple languages and cultures.

Assessment of Bullous Pemphigoid Disease Area Index during Treatment: A Prospective Study of 30 Patients

Background: Recently, a consensus Bullous Pemphigoid Disease Area Index (BPDAI) was proposed to measure therapeutic outcomes in bullous pemphigoid (BP). Objective: To compare BPDAI with other clinical parameters of disease activity at baseline and to describe the variations of BPDAI during the initial phase of treatment. Methods: Thirty BP patients were included and followed for 1 year. BPDAI was assessed at baseline and on days 30, 90 and 360 by the same investigator. Concomitantly, the number of daily new blisters, the skin surface area of erythematous/eczematous/urticarial plaques and blisters/erosions, total lesion area (TLA), pruritus score and mucosal involvement were recorded. Results: At baseline, BPDAI was 46.7 ± 25 (mean ± SD); it was well correlated with erythematous/eczematous/urticarial skin surface (r = 0.63), TLA (r = 0.83), number of daily new blisters (r = 0.7; p ≤ 0.0002) and anti-BP180 autoantibodies (r = 0.49; p = 0.006), but not with anti-BP230 autoantibodies. For the 8 patients with severe BP at baseline, the mean BPDAI was 76.5, versus 35.9 for moderate BP (p = 0.0007). A value of 56 was proposed as a cut-off value for severe BP. BPDAI decreased to 11.9 ± 8.7, 10.7 ± 12.7 and 2.5 ± 4.1 on days 30, 90 and 360, respectively. Conclusion: BPDAI rapidly decreased during the early treatment stage of BP with variations almost totally conditioned by the skin activity component.

Association of Autoantibodies to BP180 with Disease Activity in Greek Patients with Bullous Pemphigoid

39 bullous pemphigoid (BP) patients were studied to assess the clinical significance of anti-BP180 and anti-BP230 circulating autoantibodies of BP and correlate their titers with the clinical scores of the BP Disease Area Index (BPDAI) and the Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) as well as with the intensity of pruritus measured by the BPDAI pruritus component. All parameters were evaluated by the time of diagnosis (baseline), month 3, and month 6. Titers of anti-BP180 autoantibodies were strongly correlated with BPDAI (r = 0.557, P value < 0.0001) and ABSIS (r = 0.570, P value < 0.0001) values, as well as with BPDAI component for the intensity of pruritus (rho = 0.530, P value = 0.001) at baseline. At month 3, titers of anti-BP180 autoantibodies were strongly correlated with BPDAI (rho = 0.626, P value = 0.000) and ABSIS (rho = 0.625, P value = 0.000) values, as well as with the BPDAI component for the intensity of pruritus (rho = 0.625, P value = 0.000). At month 6, titers of anti-BP180 autoantibodies were strongly correlated with BPDAI (rho = 0.527, P value = 0.001) and ABSIS (rho = 0.526, P value = 0.001) values, as well as with the BPDAI component for the intensity of pruritus (rho = 0.525, P value = 0.001). There was no statistically significant correlation between titers of anti-BP230 autoantibodies and the BPDAI, ABSIS, and BPDAI component for the intensity of pruritus at the same time points.