maede rayati damavandi
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BackgroundPemphigus vulgaris (PV) is an autoimmune bullous disease of the skin and mucous membranes caused by activation and proliferation of T cells, production of Th2 cytokine profile and pathogenic antibodies. Vitamin D is a probable immunodeviator to Th2, which its actions are mediated through the vitamin D receptor (VDR). FokI is the only single nucleotide polymorphism (SNP) leading to VDR protein with a different structure and function. For the first time, we focused on FokI VDR SNP to evaluate its potential role in the genetic susceptibility to PV, particularly in the Iranian population that has a high prevalence of pemphigus.MethodsIn this case-control study, DNA samples of 122 PV patients and 233 healthy controls were extracted, and FokI genotyping was performed using the PCR-RFLP method.ResultsThe mean allele frequencies of F and f alleles in the PV and control groups were 75% and 25%, and 78% and 22%, respectively, showing no significant difference. The genotype frequencies for FF, Ff, and ff genotypes in the case group were 57.4%, 35.2%, and 7.4%, respectively. In the control group, the frequencies were 60%, 36%, and 4%, respectively. Statistical analysis showed no significant difference between the two groups.ConclusionThe present study concluded the frequencies of F and f alleles as approximately 77% and 23% in the gene pool of the Iranian population. Additionally, it showed no association between the FokI alleles and PV in this population.Keywords: FokI, pemphigus, Polymorphism, Vitamin D receptor
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BackgroundChronic graft versus host disease (cGVHD) is a major cutaneous complication of bone marrow transplantation (BMT). Although milder forms of this process may be associated with a lower incidence of tumor recurrences, it is mandatory to develop a more efficient and less harmful therapeutic approach.MethodsThis case-series study enrolled 7 patients diagnosed with chronic mucocutaneous GVHD. We divided the patients into three major categories based on the type of skin lesions: sclerodermoid, lichenoid, and mixed. Patients received several packs of narrow band UVB (NBUVB) phototherapy. Each pack contained ten sessions of NBUVB (311 nm) with a duration of at least ten seconds and a fixed radiation dosage (6 mj/cm2) during the treatment.ResultsThere were 3 patients diagnosed with lichenoid skin lesions, 2 with sclerodermoid lesions, and 2 had mixed cGVHD lesions. During the follow up period one patient was excluded due to a lower respiratory tract infection. The mean response ratio was 42% with a mean satisfaction level of 5.5 out of 10. The lichenoid group had the best, most rapid response. There were no serious adverse effects reported.ConclusionNarrow band UVB phototherapy is useful as an adjuvant therapeutic modality in cutaneous lichenoid and intraoral cGVHD with no serious adverse effects.Keywords: chronic graft versus host disease, phototherapy, narrow band ultraviolet B, bone marrow transplantation
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ایکتیوزها گروهی از بیماری ها با اختلال کراتینیزاسیون و ضایعات پوسته دار گسترده با درجات متغیری از درگیری هستند. بر پایه دانسته های ما همزمانی ایکتیوز و درماتوفیتوز که هر دو اختلالات شایعی هستند بسیار نادر است. در این گزارش ما مرد جوانی با ایکتیوز مادرزادی معرفی می کنیم که در بررسی هیستوپاتولوژیک بیوپسی های متعدد پوستی و اسمیر مستقیم، قارچ درماتوفیت PAS مثبت یافت شد. این بیمار مدت ها به خاطر پچ و پلاک های اریتماتوی گسترده تحت درمان با استروئید موضعی نیز بوده است.
کلید واژگان: درماتوفیتوز, ایکتیوز مادرزادی, اریترودرمیIchthyosis is defined as a group of diseases with keratinization disorder and diffuse scaling with highly variable degree of involvement. According to our knowledge, coincidence of ichthyosis and dermatophytosis, which both are very common disorders, is a very rare event. We report a young man with congenital ichthyosis that histological analysis of his skin biopsies and direct smear revealed PAS positive fungi. He had used topical steroids for generalized scaling erythematous patches and plaques for long time.
Keywords: dermatophytosis, congenital ichthyosis, erythroderma -
BackgroundA common Human Leukocyte Antigen (HLA) class II allele, DQβ1*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.ObjectiveTo investigate the association of HLA class II alleles and haplotypes with BP in Iranian population.MethodsFifty 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.ResultsClass II DQA1 and DQB1 typing showed a significantly higher frequency of HLA-DQA1*05:01 (45% vs. 33%, p=0.03), HLA-DQB1*03:01 (36% vs. 23.6%, p=0.02) and HLA-DQB1*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*08:01/DQA1*05:01/DQB1*03:01 (3% vs. 0%, p=0.02) haplotype showed an increase among patients compared with controls.ConclusionOur data suggest that Iranian patients with BP present the same genetic predisposition linked to HLA-DQB1*03:01 previously reported in Caucasians
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