Association between HLA-DQB1[asterisk]03:01 and Bullous pemphigoid in Iranian patients

A common Human Leukocyte Antigen [HLA] class II allele, DQ beta 1[asterisk]03:01, seems to be associated with Bullous pemphigoid [BP] in Caucasians whereas previous studies in other ethnic groups showed other HLA class II alleles as genetic predisposing factors for BP. To investigate the association of HLA class II alleles and haplotypes with BP in Iranian population. Methods: Fifty patients with Bullous pemphigoid and 180 geographically matched, healthy individuals as control group enrolled into this study. HLA typing of class II [DR and DQ alleles] was carried out using polymerase chain reaction based on sequence-specific primers method. Class II DQA1 and DQB1 typing showed a significantly higher frequency of HLA-DQA1[asterisk]05:01 [45% vs. 33%, p=0.03], HLA-DQB1[asterisk]03:01 [36% vs. 23.6%, p=0.02] and HLA-DQB1[asterisk]04:01 [4% vs. 1.6%, p=0.04] in the BP patients compared with controls. For DRB1 allele frequencies, there were no significant disease associations. The frequency of DRB1[asterisk]08:01/DQA1[asterisk]05:01/DQB1[asterisk]03:01 [3% vs. 0%, p=0.02] haplotype showed an increase among patients compared with controls. Our data suggest that Iranian patients with BP present the same genetic predisposition linked to HLA-DQB1[asterisk]03:01 previously reported in Caucasians

Pemphigus vulgaris activity score and assessment of convergent validity

Pemphigus is a rare autoimmune blistering disease with different phenotypes. The evaluation of therapeutic interventions requires a reliable, valid and feasible to use measurement. However, there is no gold standard to measure the disease activity in clinical trials. In this study we aimed to introduce the pemphigus vulgaris activity score [PVAS] measurement and to assess the convergent validity with the experts' opinion of disease activity. In PVAS scoring, the distribution of pemphigus vulgaris antigen expression in different anatomical regions is taking in to account with special consideration of the healing process. PVAS is a 0-18 scale, based on the extent of mucocutaneous involvement, type of lesion and the presence of Nikolsky's sign. The sum of the scores of total number of lesions, number of different anatomic regions involvement and Nikolsky's sign is weighted by the type of lesion. In the present study, PVAS was assessed in 50 patients diagnosed with pemphigus vulgaris by one dermatologist. Independently, five blinded experts scored all the patients through physician's global assessment [PGA]. The convergent validity with experts' opinion was assessed. The Spearman coefficient of correlation showed the acceptable value of 0.751 [95%CI: 0.534- 0.876]. PVAS is a valid, objective and simple-to-use scoring measurement. It showed a good correlation with PGA of pemphigus disease activity in Iranian patients with pemphigus vulgaris

Bullous Pemphigoid in Iranian patients: a descriptive study on 122 cases

Bullous pemphigoid is an immunobullous disease with high mortality and morbidity. Different aspects and characteristics in the patients vary in different areas in the world. Our objective was to study clinical and demographic characteristics of bullous pemphigoid in Iranian patients. In a retrospective descriptive study, we reviewed 122 patients with bullous pemphigoid within 1987-2007. Demographic characteristics, clinical manifestations, treatment, relapses and outcome were evaluated. The mean age of 122 patients was 65 +/- 18.11 years including 35.2% male and 64.8% female. The most common manifestations were cutaneous bullae [97.5%]. 27% had oral lesions. 30.3% had eosinophillia. 90 patients [73.8%] received oral prednisolone, 29 patients [23.8%] topical steroid, 2 patients tetracycline and 1 patient dapsone. 89 patients were followed after admission. Out of them 44 patients experienced first relapse and 22 patients second relapse. 41 cases [46%] were completely controlled. 11 cases [12%] were not controlled. Clinical and general characteristics of bullous pemphigoid patients differ in various regions in the world

[Comparison of the frequency of antinuclear antibodies in patients with pemphigus vulgaris and a control group]

Pemphigus vulgaris [PV] is an autoimmune blistering disease which is specific for skin and mucosal membranes. Its association with connective tissue diseases has already been reported. Considering this association, presence of Anti Nuclear Antibodies [ANAs] in PV patients will not be surprising. The aim of this study was to compare the frequency of ANA positive cases in patients suffering PV with a control group. In this case-control study, the cases were selected from the patients with PV whom were hospitalized at Razi Hospital, Tehran. The controls were chosen from patients who did not have PV. The data were collected using a questionnaire, which was designed for gathering information on participants' age, sex, PV phenotypes, and the result of ANA test. ANA positivity was assessed using indirect immunofluorescence, HEP2. In 8 [26.7%] of 30 PV patients and 3 [10.0%] of 30 controls ANA was positive [P=0.095]. The most common ANA positive patterns among cases and controls were homogeneous and speckled patterns, respectively [P=0.26]. Although in this study the frequency of positive ANA result among controls was similar to what were reported in previous studies, the frequency of this finding among PV patients was different from the previous reports. Differences in the sensitivity of the laboratory kits used in different studies as well as in the threshold for ANA positivity, and differences in the patients' eligibility criteria in different studies may explain the observed discrepancies. Clinical follow up of the PV and requesting an ANA test in the case of appearance of the signs of connective tissue diseases is recommended

Desmoglein ELISA in the diagnosis of pemphigus and its correlation with the severity of pemphigus vulgaris

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<0.05]. Desmoglein 3 index values increased with oral severity although this was not statistically significant. Iranian patients similar to Indian patients had higher positive anti-desmoglein 1 autoantibodies. Desmoglein-ELISA test is appropriate in the diagnosis of pemphigus. Desmoglein 1 index value is statistically correlated with the severity of pemphigus vulgaris