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Anti-desmoglein 3 and 1 autoantibodies are involved in the pathogenesis of pemphigus diseases. Our objective was to assess the value of ELISA in the diagnosis of pemphigus and its correlation with the severity of pemphigus vulgaris. Based on clinical presentation and histopathologic confirmation for the diagnosis of the pemphigus, 38 patients took part in the study. Sera of the patients were tested by desmoglein 1 and desmoglein 3 ELISA. Also, direct immunofluorescence was performed for all patients which revealed positive results in 36 patients (94.7%). ELISA was positive in 37 of 38 pemphigus patients (Sensitivity: 97.3%). The relationship between desmoglein 1 index values and skin severity was statistically significant (p

Background: Studies on the interaction of pemphigus and pregnancy are limited to case reports and small case series. Pregnancy is not rare in Iranian pemphigus patients due to lower age at onset of the disease. Aim: We sought to investigate the outcome of pregnancy and the course of pemphigus in pemphigus patients in a retrospective study. Methods: The files of 779 pemphigus women younger than 50 presenting to our center from 1984 till 2006 were examined for any mention of pregnancy. Data related to outcome of pregnancy and the course of the disease were collected. Results: Sixty-six patients with a history of pregnancy were identified. Forty-eight known pemphigus patients experienced one or more pregnancies during their disease (total pregnancy number: 52). The course of pemphigus was as follows in this group: 28 cases (54%) of exacerbation, 15 cases (31%) with no alteration, and 9 cases (17%) showing improvement. The rate of abortion was 9.6% (5 cases). In 18 cases, the disease had started during pregnancy, 2 of them (11%) ended in an abortion. Overall, postpartum flare was seen in 33 cases (47.1%). Conclusion: Pemphigus may be exacerbated during or after pregnancy, but often to a mild degree. Although the rate of stillbirth was not as high as previously reported, the rate of abortion was considerable. Pregnancy may have an uneventful course, especially in patients in clinical remission; nevertheless, careful monitoring of the high risk mother and fetus is mandatory. [ABSTRACT FROM AUTHOR]

Background: Pemphigus vulgaris (PV) is a rare but seriously disabling disorder of the skin and mucous membranes that can gravely impact the quality of life (QoL) of patients., Objective: The aim of the present study was to identify how family members of patients with PV are affected by the disease., Methods: A total of 118 patients with confirmed PV and one of their family members (caregivers) were enrolled in the study. To calculate disease severity, the Autoimmune Bullous Skin Disorder Intensity Score was used. The Persian version of the Dermatology Life Quality Index (DLQI) questionnaire was used to evaluate the QoL of patients and the Family Dermatology Life Quality Index (FDLQI) to evaluate the QoL of caregivers., Results: The mean age of patients was 43.14 ± 12.5 years. Ninety patients (76.3%) were female. Eighty-one patients (68.6%) had the mucocutaneous phenotype and 37 cases (31.4%) the mucosal phenotype. The DLQI score was 10.1 ± 7.1 for patients. The DLQI score was higher for patients with the mucocutaneous phenotype (11.8 ± 7.5) than those with the mucosal phenotype (6.4 ± 4.9; p  < .001). QoL was significantly affected by disease severity. FDLQI score was 13 ± 7 for caregivers, and was significantly higher in older caregivers and married ones. There was a positive correlation between patients' admission frequencies and FDLQI score. FDLQI score was also significantly affected by the Autoimmune Bullous Skin Disorder Intensity Score of patients' disease severity. The QoL of patients and their caregivers showed a significant positive correlation., Conclusion: The QoL of patients and their families are impaired significantly, and is considerably prominent in the mucocutaneous phenotype of PV and more severe forms., Limitation: Pemphigus Disease Area Index (PDAI) and Autoimmune Bullous Disease QoL (ABQoL) were not used in this study., (© 2020 Published by Elsevier Inc. on behalf of Women's Dermatologic Society.)

Background: Autoimmune bullous diseases are a group of rare, chronic, blistering diseases, with pemphigus vulgaris (PV) being the most common type in Iran. Skin and mucosal involvement and therapy may have a dismal impact on the quality of life of affected patients., Objective: We aimed to assess the validity and reliability of the Farsi (Persian) version of the Autoimmune Bullous Quality of Life (ABQoL) index in Iranian patients with PV., Methods: Forward and backward translation procedures were used to prepare the Persian version of the ABQoL questionnaire. A total of 180 patients were asked to complete the questionnaires, and 24 cases repeated the test after 2 weeks. For validity and reliability, an exploratory factor analysis was performed along with a parallel analysis to determine the number of factors. The multi-trait, multi-method matrix assessed convergent and discriminant validity. To evaluate internal reliability and reliability over time, Cronbach's alpha and intraclass correlation coefficients were reported., Results: Two factors explained a total variance of 54.9% in the data. The first and second factors comprised questions 1-3, 5, 7, 9, and 12-17 (symptoms-social) and questions 4, 6, 8, 10, and 11 (mucosal-psychological), respectively. The internal reliability of the Persian version of the ABQoL index was also acceptable, with a Cronbach's alpha of .855 for total items, .918 for the first factor, and .6 for the second factor. Our results suggest an acceptable convergent and discriminant validity of the Persian version of the ABQoL questionnaire., Conclusion: The Persian version of ABQoL index is a valid and reliable tool to monitor quality of life in patients with PV. Further studies are needed to assess the sensitivity of this instrument to changes in different disease activity and correlation with more general tools for the measurement of quality of life., (© 2020 The Authors.)

Objective: Behcet's disease (BD), as a vasculitis, can affect small and large vessels. As dermatoscopy has been shown to improve the accuracy in diagnosis of various skin lesions especially vascular patterns, we set this study to find if there is any characteristic pattern in the dermatoscopy of Behcet's mucocutaneous lesions., Methods: This prospective cross-sectional observational study designed to evaluate dermatoscopic features of Behcet's mucocutaneous lesions. Fifty six consecutive patients presenting at the outpatient clinic of the BD Research Unit were included. If present, for each patient one oral, one skin and one genital lesion were evaluated by dermatoscopy. When indicated, pathergy test was performed according to a standard protocol and the results were evaluated by dermatoscopy., Results: A total of 40 oral, 8 genital, 14 skin lesions and 14 pathergy tests were evaluated by dermatoscopy. While vascular component was the most prominent feature in oral aphthae, this component was less prominent in genital lesions. Dot vessels were the most common form of vessels in both oral and skin lesions. All the oral lesions were characterized by a central white structureless area. Skin lesions were characterized by a red structureless background. In pathergy tests, negative pricks showed absence of specific features while positive pricks were characterized by a structureless background in pink, purple or red. No obvious vascular component was detected in any of the pricks., Conclusion: It seems that these findings have no specific clues for the diagnosis of BD, but our study is the first study in this field and the findings may give way to further investigations.

Objective: To analyze Behcet's Disease (BD) in Iran, from 1975 to 2018, and compare to 35 large/small reports from other countries. Methods: Patients from all over Iran, when suspected, were sent to the BD Unit. The diagnosis was done by expert opinion. All data were recorded in the BD registry (updated in each follow-up). The data are given in percentage with 95% confidence Intervals. Results: The mean age at onset was 25.6 years. Standard deviation (SD) was 9.8. The mean disease duration was 11.7 years (SD: 8.9). Males were 55.8% (54.7-56.9), Females 44.2% (43.1-45.3), Oral Aphthosis (OA) 97.5% (97.1-97.9), genital aphthosis (GA) 64.4% (63.3-65.5), skin lesions 62.2% (61.1-63.3), ocular lesions 55.6% (54.5-56.7), Joint Manifestations 38.1% (37.0-39.2), Gastrointestinal 6.8% (6.2-7.4), Vascular 8.9% (8.3-9.5), neurological (central-peripheral) 3.9% (3.5-4.3), epididymitis 4.6% (4.1-5.1). Lab tests were positive pathergy test 50.4% (49.3-51.5), elevated ESR 51.1% (50.0-52.2), abnormal urinalysis 13.4% (12.6-14.2). The International Study Group (ISG, 1990) criteria and the International Criteria for Behcet's Disease (ICBD, 2014) had respectively a sensitivity of 76.2% (75.2-77.2) and 96.6% (96.2-97.0). The specificity was 99.3% (99.1-99.5) and 97.3% (96.9-97.7). The accuracy was 86.4% (85.8-87.0) and 96.9% (96.6-97.2). Conclusion: The most frequent manifestations were OA, GA, skin manifestations, and ocular manifestations.

Pemphigus vulgaris (PV) is an autoimmune blistering disease affecting the skin and/or mucosa. Rituximab (RTX) has been approved recently by US FDA as an effective and safe treatment of PV. The high incidence of PV in Iran encouraged our team to prepare a consensus guideline for RTX administration based on literature review and a decade experience of an expert panel. RTX is recommended for the treatment of new cases of PV as well as patients not responding to conventional therapy. Contraindications include history of anaphylaxis or IgE-mediated hypersensitivity to murine proteins of RTX, severe active infections, pregnancy, breastfeeding, severe heart failure, and arrhythmia. Prophylactic antiviral therapy is recommended in patients at risk of reactivation of HBV and isoniazid for those at risk of reactivation of tuberculosis. Concomitant use of systemic corticosteroids is recommended as a rule. Except for methotrexate, the combination with other immunosuppressive drugs is discouraged. Intravenous immunoglobulin is recommended for those at risk of infections or with extensive disease. The recommended dosage of RTX for the first cycle is 2 g either 500 mg weekly or 1 g biweekly. There is no general consensus whether the next doses of RTX be administered upon relapse or as maintenance therapy. We strongly recommend RTX sooner in the course of pemphigus., (© 2019 Wiley Periodicals, Inc.)

Objectives: This study proposed to report the characteristics of paediatric Behçet's disease (PED-BD) in a cohort of patients from Iran's registry and compare them with different reports throughout the world., Methods: From a cohort of 7504 Iranian patients with Behçet's disease those diagnosed before the age of 16 years were included in this study. Data were collected on a standard protocol comprising 105 items, including demographic features, type of presentation, and different clinical and laboratory findings., Results: PED-BD was seen in 2.7% of patients. The male/female ratio was 1.02/1, and the mean age at onset was 10.5±3.4. Positive familial history was present in 9.9%. As a first manifestation, oral aphthosis was the most frequent (75%) followed by ocular lesions in 19.1%. The prevalence rates of various manifestations were as follows: oral aphthosis: 91.7%; genital ulcer: 42.2%; skin: 51.5% (pseudofolliculitis: 43.1%, erythema nodosum: 10.3%); ocular lesions: 66.2% (anterior uveitis 52%, posterior uveitis 58.3%, retinal vasculitis 39.7%); articular manifestations: 30.9%; neurological involvement: 4.9%; vascular involvement: 6.4% (venous 4.9%, arterial 2.5%); gastrointestinal manifestations: 5.9%; epididymo-orchitis: 8.7% (boys); high ESR (≥20): 50.8%; abnormal urine: 14.1%; positive pathergy test: 57%; HLA-B5/51: 48.7%. ICBD criteria have the highest sensitivity for the classification of PED-BD patients in Iran (91.7%)., Conclusions: The clinical spectrum of PED-BD in Iran in this study was similar to that of other reports; however, genital ulcers, skin lesions (notably erythema nodosum), and gastrointestinal involvement were noticed to occur less frequently, while ocular lesions were more frequent and more severe compared to other reports.