Family impact of pemphigus disease in an Iranian population using the Family Dermatology Life Quality Index.

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.

Autoimmune Bullous Disease Quality of Life (ABQoL) questionnaire: Validation of the translated Persian version in pemphigus vulgaris.

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.

Iranian guideline for rituximab therapy in pemphigus patients.

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.

Behcet's disease in Iran: Analysis of 7641 cases.

To analyze Behcet's Disease (BD) in Iran, from 1975 to 2018, and compare to 35 large/small reports from other countries. Patients from all over Iran, when suspected, were sent to the BD Unit. The diagnosis was done by expert opinion.

Paediatric Behçet's disease in Iran: report of 204 cases.

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.

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.

Evaluation of Autoimmune Bullous Diseases in Elderly Patients in Iran: A 10-Year Retrospective Study.

Autoimmune bullous diseases (ABDs) are uncommon but significant skin disorders with relatively high morbidity and mortality. Some surveys have been carried out to describe the spectrum of ABDs in a region, but this is the first that has focused on ABDs in elderly patients. This study was conducted to determine the clinicoepidemiologic features of ABDs in elderly patients.

Zeitlicher Verlauf der avaskulären Nekrose des Hüftkopfes bei Patienten mit Pemphigus vulgaris.

Hintergrund Und Ziele: Pemphigus vulgaris (PV) wird in der Regel mit systemischen Corticosteroiden und Immunsuppressiva behandelt. Avaskuläre Nekrose (AVN) des Hüftkopfes ist eine gut bekannte schwerere Komplikation einer Corticosteroid-Therapie. Die Charakteristika dieser schweren Komplikation bei PV sind nach wie vor unbekannt.

Temporal course of avascular femoral head necrosis in patients with pemphigus vulgaris.

Background And Objectives: Pemphigus vulgaris (PV) is typically treated with systemic corticosteroids and immunosuppressive agents. Avascular necrosis (AVN) of the femoral head is a well-recognized major complication of corticosteroid therapy. The characteristics of this serious complication in PV remain unknown.

Neurological diseases and bullous pemphigoid: A case-control study in Iranian patients.

Introduction: Neurological diseases are important co-morbidities found in association with bullous pemphigoid. Various neurological conditions (stroke, Parkinson's disease, dementia, epilepsy and multiple sclerosis) have been reported as associations of this bullous disease; whether these are significant has not been definitely proved. However, the presence of neurological conditions is a predictor of poorer prognosis.

Behcet's disease: epidemiology, clinical manifestations, and diagnosis.

Behcet's Disease (BD) is classified among vasculitides. The aim of this review was to put together different known reports in order to help the reader to better understand the disease, to avoid the frequent misdiagnosis, and to decide the best treatment. Areas covered: a) Epidemiology: BD is rare, and is seen along the Silk Road, from 20 to 420/100,000 in Turkey and 80/100,000 in Iran, to 0.

Immunologic prediction of relapse in patients with pemphigus vulgaris (PV) in clinical remission.

Background: Pemphigus vulgaris (PV) is characterized by multiple relapses, occurring especially in patients on minimal therapy or off therapy.

Objective: To identify immunologic predictors (anti-desmoglein [Dsg] 1 and 3 antibodies; direct immunofluorescence [DIF]) for relapse in PV patients.

Methods: Eighty-nine patients in complete clinical remission for at least 6 months and receiving less than or equal to 10 mg prednisolone daily and no immunosuppressive drugs were evaluated using DIF (n=89) and Dsg ELISA (n=46).

Trauma-induced pemphigus: a case series of 36 patients.

Background And Objectives: Pemphigus is a group of autoimmune diseases characterized by intraepidermal acantholytic blisters. Isomorphic responses, or Koebner phenomenon (KP), defined as the appearance of typical lesions of a disease following trauma are rarely reported in pemphigus. Our aim was to present patients who developed new pemphigus lesions as a result of skin trauma.

Adult Behcet's disease in Iran: analysis of 6075 patients.

Introduction: This is an analysis of adult Behcet's disease (BD) in Iran, from the Iran Registry of 7187 BD patients, gathered from 1975 to 2014, among which 6075 were adults (84.5%).

Patients: Patients were seen by a multidisciplinary team of experts.

The saga of diagnostic/classification criteria in Behcet's disease.

There are 17 sets of diagnosis/classification criteria for Behcet's disease: Curth (1946), Hewitt (1969), Mason (1971), Japan (1972), Hubault (1974), O'Duffy (1974), Cheng (1980), Dilsen (1986), Japan revised criteria (1988), International Study Group on Behcet's disease (ISG criteria, 1990), Iran traditional criteria (1993), Iran Classification Tree (1993), Dilsen revised criteria (2000), Korea Criteria (2003), International Criteria for Behcet's Disease (ICBD, 2006) and the revised ICBD (2010). This review is intended to show how to use them and show their performance in patients from different parts of the world. The major sets of patients (patient numbers, control numbers, year) on which the criteria were tested are: ISG set (886/97/1990), Iran (2069/1540/1993), Asia and Pacific League of Associations for Rheumatology (APLAR: 216/145/1998), Russia (105/233/2000), USA (50/NA/2000 [NA: not available]), India (50/NA/2004), Singapore (37/NA/2004), China (98/NA/2004), Korea (1454/NA/2004), Iran (4900/2020/2004), ICBD (2556/1163/2006), Germany (86/38/2008), China (322/118/2008), Iran (6128/3400/2010) and Iran (7011/5226/2013).

Osteoporosis in patients with Pemphigus Vulgaris before steroid therapy.

Pemphigus Vulgaris (PV) is often complicated by osteoporosis. Although corticosteroid therapy undoubtedly plays a causative role, inflammation associated with PV may also contribute to osteoporosis. This study was designed to determine the prevalence of osteoporosis in patients with PV before corticosteroid therapy and to compare these findings with those reported previously in healthy volunteers.

Lipoid proteinosis: phenotypic heterogeneity in Iranian families with c.507delT mutation in ECM1.

Lipoid proteinosis (LP) is a rare autosomal recessive genodermatosis caused by loss-of-function mutations in the ECM1 gene, and previous studies have noted phenotypic variability. In this study, we examined 12 patients representing three Iranian families for clinical manifestations and genotyped them for mutations in ECM1. LP was diagnosed with characteristic mucocutaneous and neurologic manifestations.

Assessment of the therapeutic benefit of oral prednisolone and common adjuvant therapy in stage II of randomized controlled trial study for management of pemphigus vulgaris.

Background: Pemphigus is an autoimmune blistering mucocutaneous disorder. Common treatments include corticosteroids and immunosuppressive drugs. This study aimed to assess the therapeutic effects of oral prednisolone along with the common adjuvant therapy in pemphigus vulgaris.

Genital aphthosis in Behçet's disease: is it associated with less eye involvement?

Behçet's disease (BD) is a multisystem disease classified among the vasculitides with various clinical features. Genital aphthosis (GA) is one of the major manifestations of BD. The aim of this study was to evaluate the characteristics of BD patients with GA.

Pemphigus disease activity measurements: pemphigus disease area index, autoimmune bullous skin disorder intensity score, and pemphigus vulgaris activity score.

Importance: Recently, the clinical pemphigus disease activity indexes of Pemphigus Disease Area Index (PDAI), Autoimmune Bullous Skin Disorder Intensity Score (ABSIS), and Pemphigus Vulgaris Activity Score (PVAS) were validated to correlate with physician global assessment. The antidesmoglein (anti-Dsg) autoantibodies are known to correlate mostly with pemphigus disease activity. The correlation between these indexes and anti-Dsg1 and anti-Dsg3 enzyme-linked immunosorbent assay values has not been previously evaluated.