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Abstract Background: Pemphigus constitutes a group of autoimmune bullous diseases. A reduction in the incidence of endemic pemphigus foliaceus and an increase in pemphigus vulgaris has been described, but there are no studies in Minas Gerais that address the subject. Objective: To describe the epidemiological and clinical profile of patients with pemphigus treated at the Dermatology Service of a public University Hospital in the state of Minas Gerais, Brazil. Methods: An observational, descriptive, and cross-sectional study was carried out of cases of endemic pemphigus foliaceus and pemphigus vulgaris, for a period of six months. A questionnaire was filled out with epidemiological and clinical data on the disease. Results: A total of 122 patients were included in the study, 64 with endemic pemphigus foliaceus and 58 with pemphigus vulgaris. When comparing patients with endemic pemphigus foliaceus and those with pemphigus vulgaris, a statistical difference was observed between the median age of initial disease manifestation (p = 0.001), patient occupation (p = 0.010), area of residence (p = 0.000), forests (p = 0.000) and rivers/streams close to the dwelling (p = 0.001) and the number of systemic medications required to control the disease (p = 0.002). When comparing patients with endemic pemphigus foliaceus to those evaluated in a study carried out at the same service in 2008, there was a statistical difference in the area of residence (p = 0.030). Study limitations: The assessed population comes from a tertiary care service that is not a reference for the entire state. Conclusions: Patients with endemic pemphigus foliaceus and pemphigus vulgaris maintain statistically significant differences regarding their main variables in the literature, such as age and area of residence. Historically, there has been a reduction in cases of endemic pemphigus foliaceus and an increase in cases of pemphigus vulgaris in this population.

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BACKGROUND: Pemphigus constitutes a group of autoimmune bullous diseases. A reduction in the incidence of endemic pemphigus foliaceus and an increase in pemphigus vulgaris has been described, but there are no studies in Minas Gerais that address the subject. OBJECTIVE: To describe the epidemiological and clinical profile of patients with pemphigus treated at the Dermatology Service of a public University Hospital in the state of Minas Gerais, Brazil. METHODS: An observational, descriptive, and cross-sectional study was carried out of cases of endemic pemphigus foliaceus and pemphigus vulgaris, for a period of six months. A questionnaire was filled out with epidemiological and clinical data on the disease. RESULTS: A total of 122 patients were included in the study, 64 with endemic pemphigus foliaceus and 58 with pemphigus vulgaris. When comparing patients with endemic pemphigus foliaceus and those with pemphigus vulgaris, a statistical difference was observed between the median age of initial disease manifestation (p = 0.001), patient occupation (p = 0.010), area of residence (p = 0.000), forests (p = 0.000) and rivers/streams close to the dwelling (p = 0.001) and the number of systemic medications required to control the disease (p = 0.002). When comparing patients with endemic pemphigus foliaceus to those evaluated in a study carried out at the same service in 2008, there was a statistical difference in the area of residence (p = 0.030). STUDY LIMITATIONS: The assessed population comes from a tertiary care service that is not a reference for the entire state. CONCLUSIONS: Patients with endemic pemphigus foliaceus and pemphigus vulgaris maintain statistically significant differences regarding their main variables in the literature, such as age and area of residence. Historically, there has been a reduction in cases of endemic pemphigus foliaceus and an increase in cases of pemphigus vulgaris in this population.

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BACKGROUND: Systematic reviews of Randomized Controlled Trials (RCTs) are considered high-level evidence to support a decision on therapeutic interventions, and their methodological quality is essential to provide reliable and applicable results. OBJECTIVE: This meta-epidemiological study aimed to map and critically appraise systematic reviews assessing treatments for vesiculobullous skin diseases. METHODS: We conducted a comprehensive search strategy on MEDLINE (via Pubmed) in December 2022 without restrictions to find systematic reviews evaluating pharmacological interventions for vesiculobullous skin diseases. The methodological quality was assessed using the AMSTAR-2 tool, and additional information was extracted. We identified nine systematic reviews published between 2002 and 2021, seven assessing pemphigus. RESULTS: According to the AMSTAR-2 tool, 55.6% were classified as critically low quality, 22.2% as moderate quality, 11.1% as low and 11.1% as high quality. No review assessed the certainty of the evidence (GRADE); 86% of pemphigus reviews had at least two overlapping RCTs. There were some limitations regarding methodological flaws and the AMSTAR-2 tool use CONCLUSIONS: These findings reveal a frail methodological quality of systematic reviews about vesiculobullous diseases treatment that may impact the results. Therefore, methodological rigor is mandatory for future systematic reviews to avoid duplication of effort and increase the certainty of the evidence supporting decision-making.

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Objective:To investigate the current status of multidrug-resistant bacteria (MDRO) wound infections in patients with autoimmune bullous diseases (AIBDs), and to analyze their risk factors.Methods:A retrospective study was conducted, and inpatients with AIBDs accompanied by wound infections were collected from Hospital of Dermatology, Chinese Academy of Medical Sciences from January 2020 to December 2022. A descriptive analysis was carried out to analyze the basic characteristics of these patients and pathogenic characteristics of MDRO. Univariate and binary logistic regression models were used to analyze independent risk factors for MDRO infections in patients with AIBDs. Differences between the MDRO infection group and common bacterial infection group were analyzed by using t test, Mann-Whitney U test and chi-square test. Results:Totally, 271 patients with AIBDs accompanied by wound infections were included, including 159 males (58.7%) and 112 females (41.3%), and 142 patients (52.4%) were aged over 60 years. Most patients with AIBDs were diagnosed with pemphigus vulgaris (131 cases, 48.3%), or bullous pemphigoid (99 cases, 36.5%). Bacterial culture was positive in all the patients, and 74 (27.3%) were infected with MDRO; a total of 108 strains of MDRO were detected, with relatively high detection rates of Staphylococcus (82 strains, 75.9%) and Enterobacter (15 strains, 13.9%). Significant differences were observed between the MDRO infection group and the common bacterial infection group in the duration of hospitalization, involved body surface area, proportions of patients self-modificating drug dosage, proportions of patients topically using antibiotic ointments, proportions of patients using immunosuppressants, duration of glucocorticoid use, maximum dose of glucocorticoids and the first albumin level at admission (all P < 0.05), while there were no significant differences in the gender, age, proportions of patients at first hospitalization, types of AIBDs, duration of education, body mass index, disease duration, proportions of smoking patients, proportions of drinking patients, proportions of patients with comorbid chronic diseases, surgical history, prevalence of hypoalbuminemia, prevalence of mucosal involvement, proportions of patients receiving topical glucocorticoids, proportions of patients using biological agents, duration of antibiotic use, and the first total protein level at admission between the two groups (all P > 0.05). Logistic regression analysis showed that the use of topical antibiotic ointments, use of immunosuppressants, maximum dose of glucocorticoids, and self-modification of drug dosage were independent risk factors for MDRO infections (all P < 0.05) . Conclusions:The patients with AIBDs were prone to develop MDRO infections in wounds, and Staphylococcus infections were the most common. The use of topical antibiotic ointments, use of immunosuppressants, high dose of glucocorticoids, and self-modification of drug dosage may increase the risk of infections in patients with AIBDs.

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Abstract Background Systematic reviews of Randomized Controlled Trials (RCTs) are considered high-level evidence to support a decision on therapeutic interventions, and their methodological quality is essential to provide reliable and applicable results. Objective This meta-epidemiological study aimed to map and critically appraise systematic reviews assessing treatments for vesiculobullous skin diseases. Methods We conducted a comprehensive search strategy on MEDLINE (via Pubmed) in December 2022 without restrictions to find systematic reviews evaluating pharmacological interventions for vesiculobullous skin diseases. The methodological quality was assessed using the AMSTAR-2 tool, and additional information was extracted. We identified nine systematic reviews published between 2002 and 2021, seven assessing pemphigus. Results According to the AMSTAR-2 tool, 55.6% were classified as critically low quality, 22.2% as moderate quality, 11.1% as low and 11.1% as high quality. No review assessed the certainty of the evidence (GRADE); 86% of pemphigus reviews had at least two overlapping RCTs. There were some limitations regarding methodological flaws and the AMSTAR-2 tool use Conclusions These findings reveal a frail methodological quality of systematic reviews about vesiculobullous diseases treatment that may impact the results. Therefore, methodological rigor is mandatory for future systematic reviews to avoid duplication of effort and increase the certainty of the evidence supporting decision-making.

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BACKGROUND: Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are subtypes of pemphigus with distinct clinical and laboratory features. The transition between these two subtypes has rarely been reported previously. METHODS: The data of PV patients who exhibited clinical and immunoserological transition to PF during the follow-up period were retrospectively evaluated regarding their demographical, clinical, and laboratory characteristics. RESULTS: Among 453 patients diagnosed with PV, 13 (2.9%) patients exhibited clinical and immunoserological transition from PV to PF. The mean age of PV patients at the time of diagnosis was 39.8 ± 14.7 (19‒62) years and 7 (53.8%) of them were female. These patients showed clinical and immunoserological transition from PV to PF after a period ranging from 4 months to 13 years (mean 36.2 ± 41 months). In addition to typical clinical features of PF, all patients had positive anti-desmoglein-1 and negative anti-desmoglein-3 antibody levels after the clinical transition had occurred without any mucosal involvement. During a mean 7.8 ± 5.8 (2‒21) years of follow-up period after the transition from PV to PF, only one female patient had experienced a re-transition to PV characterized by a relapse of disease involving mucosal surfaces with positive anti-desmoglein-3 antibody levels following a 5-year period of remission period without treatment. STUDY LIMITATIONS: Single-center study with a retrospective study design. CONCLUSION: Our series is the largest group of patients reported to show the transition from PV to PF to date with a long follow-up period. The reason behind the disappearance of anti-desmoglein-3 antibodies and the pathogenesis of this phenomenon is not yet elucidated.

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Objective:To summarize clinical, histopathological, immunoserological, and therapeutic features of patients with autoimmune subepidermal bullous diseases characterized by annular erythema and blisters.Methods:Clinical data were collected from patients with autoimmune subepidermal bullous diseases characterized by annular erythema and blisters in the Hospital of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College from 2015 to 2022, and their clinical, pathological, immunoserological and therapeutic characteristics were retrospectively analyzed.Results:A total of 25 patients were enrolled, including 10 males and 15 females, aged 39.21 ± 24.65 years; there were 9 patients with linear IgA bullous dermatosis (LABD), 7 with bullous pemphigoid (BP), 5 with anti-P200 pemphigoid and 4 with epidermolysis bullosa acquisita (EBA), and 20 (80%) patients suffered from pruritus to different extents. Dermal eosinophil infiltration was observed in 15 (60%) patients, elevated peripheral blood eosinophil counts in 11 (44%), and both were observed in 7 (28%). Indirect immunofluorescence on salt-split skin and Western blot analysis showed that both IgG and IgA anti-basement membrane zone antibodies were present in 9 patients, including 4 with BP, 1 with LABD, 2 with anti-P200 pemphigoid, and 2 with EBA; and multiple antibodies against different basement membrane zone antigens were present in 5. All the 7 BP patients were treated with systemic glucocorticoids, including 5 (71.4%) receiving combined immunosuppressive agents, and 2 receiving combined minocycline; the patients with LABD, anti-P200 pemphigoid or EBA were sensitive to anti-inflammatory drugs and dapsone.Conclusion:Multiple types of autoimmune subepidermal bullous diseases may manifest as annular erythema and blisters, and it is necessary to make a differential diagnosis based on immunoserological tests.

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Objective:To analyze clinical features of coronavirus disease 2019 in patients with autoimmune blistering diseases (AIBDs), and to explore risk factors for the development of severe coronavirus disease 2019 in patients with AIBDs.Methods:Clinical data were collected from patients with AIBDs who were hospitalized in Zhongda Hospital, Southeast University from August 2021 to December 2022, and were retrospectively analyzed. Independent sample t-test, Mann-Whitney U test, or chi-square test (Fisher′s exact test) were used to analyze factors associated with severe coronavirus disease 2019 in AIBD patients. Results:A total of 30 patients with AIBDs accompanied by coronavirus disease 2019 were included, they were aged 62.90 ± 15.72 years, and included 16 males and 14 females; pemphigus vulgaris (12 cases, 40.00%) and bullous pemphigoid (13 cases, 43.33%) predominated in the disease spectrum of AIBDs in these patients. The common clinical manifestations of coronavirus disease 2019 were cough (25 cases, 83.33%), fatigue (22 cases, 73.33%), and fever (15 cases, 50.00%) ; most patients had mild clinical symptoms, while 7 (23.33%) developed into severe coronavirus disease 2019, including 2 out of 14 aged 30 - 59 years and 5 out of 16 elderly patients aged ≥ 60 years, and 2 out of the 5 elderly patients finally died. According to the clinical classification criteria of coronavirus disease 2019, the 30 patients were divided into the mild-to-moderate group (23 cases) and severe group (7 cases) ; the duration of AIBDs was significantly shorter in the severe group (10.71 ± 10.72 months) than in the mild-to-moderate group (27.61 ± 19.67 months, t = 2.16, P = 0.040) ; the remission rate of AIBDs was significantly higher in the mild-to-moderate group (21/23) than in the severe group (4/7, χ2 = 4.36, P = 0.037) ; the average daily dosage of methylprednisolone during the last month was significantly lower in the mild-to-moderate group ( M[ Q1, Q3]: 8 [4, 14] mg) than in the severe group (24 [12, 47] mg, U = 133.50, P = 0.007), and the proportion of patients treated with methylprednisolone at an average dosage of > 8 mg was significantly higher in the severe group (6/7) than in the mild-to-moderate group (8/23, χ2 = 5.59, P = 0.031) . Conclusions:Among the patients with AIBDs accompanied by coronavirus disease 2019, the incidence and mortality of severe coronavirus disease 2019 were higher in elderly patients than in young patients. The short course and no-remission of AIBDs, and the average daily dosage of methylprednisolone being>8 mg/d during the last month were risk factors for the development of severe coronavirus disease 2019 in the patients with AIBDs.

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Abstract Background Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are subtypes of pemphigus with distinct clinical and laboratory features. The transition between these two subtypes has rarely been reported previously. Methods The data of PV patients who exhibited clinical and immunoserological transition to PF during the follow-up period were retrospectively evaluated regarding their demographical, clinical, and laboratory characteristics. Results Among 453 patients diagnosed with PV, 13 (2.9%) patients exhibited clinical and immunoserological transition from PV to PF. The mean age of PV patients at the time of diagnosis was 39.8 ± 14.7 (19‒62) years and 7 (53.8%) of them were female. These patients showed clinical and immunoserological transition from PV to PF after a period ranging from 4 months to 13 years (mean 36.2 ± 41 months). In addition to typical clinical features of PF, all patients had positive anti-desmoglein-1 and negative anti-desmoglein-3 antibody levels after the clinical transition had occurred without any mucosal involvement. During a mean 7.8 ± 5.8 (2‒21) years of follow-up period after the transition from PV to PF, only one female patient had experienced a re-transition to PV characterized by a relapse of disease involving mucosal surfaces with positive anti-desmoglein-3 antibody levels following a 5-year period of remission period without treatment. Study limitations Single-center study with a retrospective study design. Conclusion Our series is the largest group of patients reported to show the transition from PV to PF to date with a long follow-up period. The reason behind the disappearance of anti-desmoglein-3 antibodies and the pathogenesis of this phenomenon is not yet elucidated.

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There are overlaps in the pathogenesis of autoimmune bullous diseases (AIBDs) and psoriasis. In recent years, there have been many reports on correlations between psoriasis and AIBDs. Bullous pemphigoid is the most common AIBD associated with psoriasis, followed by pemphigus vulgaris, pemphigus erythematosus and linear IgA bullous dermatosis. AIBDs occur after the onset of psoriasis in most cases, but simultaneously with or before the onset of psoriasis in some patients. This review summarizes correlations between AIBDs and psoriasis, as well as possible mechanisms.

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Linear IgA bullous dermatosis (LABD) is a rare autoimmune subepidermal blistering disease. Currently, researches on LABD are still limited, and most are case reports. This review summarizes research advance in etiology and pathogenesis, clinical and histopathological manifestations, diagnosis and treatment of LABD.

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Autoimmune bullous dermatoses are a heterogeneous group of diseases with autoantibodies against structural skin proteins. Although the occurrence of autoimmune bullous dermatoses during pregnancy is low, this topic deserves attention, since the immunological and hormonal alterations that occur during this period can produce alterations during the expected course of these dermatoses. The authors review the several aspects of autoimmune bullous dermatoses that affect pregnant women, including the therapeutic approach during pregnancy and breastfeeding. Gestational pemphigoid, a pregnancy-specific bullous disease, was not studied in this review.

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Objective:To summarize clinical characteristics of and treatment experience with patients with critical illnesses in a dermatological ward.Methods:All patients with serious or life-threatening conditions, who were hospitalized at the dermatological ward of the Second Xiangya Hospital of Central South University from July 9, 2011 to December 31, 2020, were collected, and their clinical data were retrospectively analyzed. Demographic characteristics, disease types and proportions, main complications, causes of serious or life-threatening conditions, important treatment measures and outcomes were summarized, and causes of death were also analyzed and discussed.Results:A total of 1 057 patients with critical illnesses were collected, with a male-to-female ratio of 1∶1.11, and 64.81% of them aged 18 to 65 years. The types of diseases mainly included drug eruptions (332 cases) , connective tissue diseases (226 cases) , bullous skin diseases (104 cases) , psoriasis (57 cases) , erythroderma (45 cases) , infectious skin diseases (67 cases) , etc. Among them, psoriasis (39 cases) and erythroderma (32 cases) mostly occurred in males, and connective tissue diseases (168 cases) mostly occurred in females. Common complications mainly involved infections, important organ damage or dysfunction, hypoalbuminemia, and fluid, electrolyte and acid-base imbalances. A total of 94 patients were diagnosed with life-threatening conditions, which were found to be mainly caused by primary skin diseases, hematologic abnormalities, respiratory failure, nervous system abnormalities, renal failure, sepsis, fluid, electrolyte and acid-base imbalances, etc. During the management of critical illnesses, 43 patients were treated with high-dose glucocorticoid pulse therapy, 264 were treated with gamma-globulin pulse therapy, 355 were transfused with other blood products, and 34 received special therapies such as hemoperfusion/immunoadsorption therapy, plasma exchange, dialysis, artificial liver support therapy; 42 patients were transferred to the intensive care unit (ICU) , 12 were transferred to the department of surgery for operations, and 12 were transferred to the department of obstetrics and gynecology for delivery or induction of labor. After treatment, 989 patients (93.57%) achieved improvement and were discharged. A total of 14 patients (1.32%) died, of whom 7 died of secondary sepsis, 2 died of severe pulmonary infections, 2 died of asphyxia caused by respiratory mucosa shedding-induced airway obstruction, the other 3 died of gastrointestinal hemorrhage, cerebral hemorrhage and neuropsychiatric systemic lupus erythematosus, respectively.Conclusions:Critical cases in the dermatological ward mainly suffered from serious skin diseases such as severe drug eruptions, connective tissue diseases and bullous skin diseases, as well as complications such as severe underlying diseases, severe organ dysfunction, sepsis or severe fluid, electrolyte and acid-base imbalances. In terms of treatment, it is of critical significance to make a clear diagnosis and assess the severity of disease as early as possible, monitor and prevent possible complications, and to consult with specialists in relevant disciplines in time.

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Abstract Hailey-Hailey disease, or familial benign pemphigus, is a rare bullous genodermatosis that usually presents with flaccid blisters, erosions, and maceration limited to flexural areas, resulting in increased morbidity and reduced quality of life for affected patients. The authors report an unusual case of generalized Hailey-Hailey disease with erythroderma and fatal outcome.

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Hailey-Hailey disease, or familial benign pemphigus, is a rare bullous genodermatosis that usually presents with flaccid blisters, erosions, and maceration limited to flexural areas, resulting in increased morbidity and reduced quality of life for affected patients. The authors report an unusual case of generalized Hailey-Hailey disease with erythroderma and fatal outcome.

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Eosinophilic spongiosis is a histological feature shared by some distinct inflammatory disorders, and is characterized by the presence of intraepidermal eosinophils associated with spongiosis. Most often, isolated eosinophilic spongiosis indicates the early stages of a subjacent autoimmune bullous dermatosis, such as the pemphigus group and bullous pemphigoid. Herein, the main causes of eosinophilic spongiosis are discussed, as well as the supplementary investigation needed to elucidate its etiology.

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Abstract Eosinophilic spongiosis is a histological feature shared by some distinct inflammatory disorders, and is characterized by the presence of intraepidermal eosinophils associated with spongiosis. Most often, isolated eosinophilic spongiosis indicates the early stages of a subjacent autoimmune bullous dermatosis, such as the pemphigus group and bullous pemphigoid. Herein, the main causes of eosinophilic spongiosis are discussed, as well as the supplementary investigation needed to elucidate its etiology.

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Abstract: Paraneoplastic pemphigus is a rare and severe autoimmune blistering disease characterized by mucocutaneous lesions associated with benign and malignant neoplasms. Diagnostic criteria include the presence of chronic mucositis and polymorphic cutaneous lesions with occult or confirmed neoplasia; histopathological analysis exhibiting intraepidermal acantholysis, necrotic keratinocytes, and vacuolar interface dermatitis; direct immunofluorescence with intercellular deposits (IgG and C3) and at the basement membrane zone (IgG); indirect immunofluorescence with intercellular deposition of IgG (substrates: monkey esophagus and simple, columnar, and transitional epithelium); and, autoreactivity to desmogleins 1 and 3, desmocollins 1, 2, and 3, desmoplakins I and II, envoplakin, periplakin, epiplakin, plectin, BP230, and α-2-macroglobulin-like protein 1. Neoplasias frequently related to paraneoplastic pemphigus include chronic lymphocytic leukemia, non-Hodgkin lymphoma, carcinomas, Castleman disease, thymoma, and others. Currently, there is no standardized treatment for paraneoplastic pemphigus. Systemic corticosteroids, azathioprine, mycophenolate mofetil, cyclosporine, rituximab, cyclophosphamide, plasmapheresis, and intravenous immunoglobulin have been used, with variable outcomes. Reported survival rates in 1, 2, and 5 years are 49%, 41%, and 38%, respectively.

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Abstract: Background: The Autoimmune Bullous Disease Quality of Life (ABQOL) and the Treatment of Autoimmune Bullous Disease Quality of Life (TABQOL) questionnaires proved to be reliable tools that measure the disease and treatment burden. Objectives: We aimed to assess the ABQOL and TABQOL in the Arabic population. Methods: The English questionnaires were translated into the Arabic language by a certified translation agency. Eighty autoimmune bullous disease (AIBD) patients were included in this study. Patients were asked to answer 2 questionnaires. After 1 week the same patients were asked to answer the same questionnaires again. Results: The age of the patients ranged from 19 to 81 years (mean=46), 19 males, 61 females. The ABQOL ranged from 0-37 (mean=16.4±9.2). The TABQOL ranged from 2-43 (mean=21.5±9.4). Test-retest reliability was acceptable, Cronbach's alpha was 0.76 for ABQOL and 0.74 for TABQOL. There was no significant correlation between the age of the patients and ABQOL, r =-0.2, p value was 0.183. There was a significant negative correlation between the age of the patients and the TABQOL, r=-0.2, p value was 0.039. There was a significant negative correlation between the education of the patients and the TABQOL, r=-0.3, p value was 0.007. Study limitations: Small sample size of some AIBDs and patients with severe disease. Conclusion: Objective and valuable measurements such as ABQOL and TABQOL are now available to help physicians understand their patient's distress and should be used in every patient with AIBD. Younger and less educated patients appear to have more effects on their QOL from the treatments.

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