Journal of

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Background and Aim: 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.
Methods: 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.
Results: 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).
Conclusion: 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.

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Background and Aim: Pemphigus is one of the most common types of autoimmune blistering disease that requires systemic immunosuppressive therapy. Immunosuppressive therapy has improved the disease outcome in recent decades, but long-term use of them has side effects. Recently, it has been tried to evaluate immunosuppressive drugs with less side effects. One of them is mycophenolate mofetil. The aim of this study was to evaluate of therapeutic efficacy of this drug in the patients with refractory pemphigus vulgaris.
Methods: Three hundred files of patients with pemphigus vulgaris were reviewed and among them 28 patients who had received mycophenolate mofetil due to resistance to treatment were entered to this study. The data were obtained from patients’ files and were analyzed using Kendall's tau-c correlation, Mann-Whitney and ANOVA tests.
Results: Male to female ratio was 1.8:1 with an average age of 43.3±13.6 year. Twenty-eight cases of pemphigus vulgaris were treated receiving 2gr/day mycophenolate mofetil + 1mg/kg/day prednisolone. Remission occurred after 3 months. Seventeen patients (10 men and 7 women) had complete remission. There was no significant association between gender and treatment response (P=0.58). There was no significant association between mean age of treated patients and untreated patients with treatment response (P=0.77). Also, there was no significant association between severity of mucosal (P=0.80) and severity of skin involvement with treatment response (P=0.80). Ten patients who received mycophenolate mofetil more than 12 months had treatment response to mycophenolate mofetil and they did not have any relapse in the follow up period.
Conclusion: Mycophenolate mofetil is effective and safe as an adjuvant therapy in patients with pemphigus vulgaris especially in refractory pemphigus. Initiation of the therapeutic effect is slow.

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Background and Aim: Pemphigus vulgaris (PV) is the most common bullous autoimmune disease, which can cause mortality and morbidity in the patients who suffer from it. Researches to find reliable noninvasive laboratory tests to diagnose and monitor PV patients are being conducted.The aim of this study is to find the sensitivity of serum and salivary anti-DSG1 and anti-DSG3 antibodies in the diagnosis of PV by ELISA and to compare the results of serum and salivary autoantibodies with each other. Methods: In this case-control study, 40 newly diagnosed patients with PV were recruited. Forty healthy controls were also recruited to this study. The clinical diagnosis of PV was confirmed by histopathology and direct immunofluorescence (DIF). Demographic data, disease severity and phenotypes were recorded on the questionnaires, which were developed for this study. DSG1 and DSG3 ELISA test were performed on serum and salivary samples of patients and controls. Results: The mean±standard deviation age of patients, 43.37±11.94, with a range of 26 to 71 years. The sensitivities of serum anti-DSG3 and anti-DSG1 were 85% (34 cases had positive test results) and 90%, (36 cases had positive test results) respectively. The sensitivities of salivary anti-DSG3 and anti-DSG1 antibodies were accordingly 42.5% (17 cases had positive test results) and 60%, (24 cases had positive test results) respectively. Conclusion: While the sensitivities of serum ELISA in detection of anti-DSG1 and anti-DSG3 were significantly higher than those of salivary ELISA, since the levels of the latter are chnaging in parallel to those of serum ELISA, they might be used to monitor the disease activity.

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Background and Aim: The standard treatment regimen for pemphigus is adminitration of systemic corticosteroids and adjuvant agents. Previous studies have shown that corticosteroid therapy may alter lipid profile and reduce the atherogenic index in some disorders. This study was conducted to evaluate the changes in lipid profile in pemphigus patients treated with oral corticosteroids alone or in combination with adjuvants.

Methods: In this retrospective corss-sectional study, medical records of 148 pemphigus patients admitted in Razi Hospital in Tehran were reviewed for serum levels of low-density lipoprotein (LDL), high-density lipoprotein (HDL), total cholestrol, and triglyceride on the day of admission till 3 weeks after initiation of the treatment.

Results: Fifty-eight percent of patients were female and the mean age of the patients was 47.1 years. 79.3% and 13.3% of patients had been treated with prednisolone plus azathiorine or prednisolone alone, respectively. The remainig patients were treated by administration of oral prednisolone and other adjuvants. Serum levels of LDL, HDL, total cholestrol and triglyceride were significantly higher 3 weeks after initiation of treatment compared to baseline (P<0.05). With the exception of the signifcantly higher level of triglycerides in women, there was no statistically significant association between the levels of other serum lipids at the end of 3 weeks treatment with gender, age, severity of the disease, use of statins, and the treatment regimen (P>0.05).

Conclusion: Although the level of different components of the lipid profile in Iranian patients with pemphigus changed after receiving treatment for 3 weeks, it seems that those changes were not associated with an increased atherogenic index of pemphigus patients.

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Background and Aim: Pemphigus vulgaris is a rare autoimmune intraepidermal blistering disease affecting the skin and mucous membranes. Copper and zinc are two necessary trace elements in the human body that have antioxidant activity in the wound healing process. The aim of this study was to determine the serumic level of zinc and copper in newly diagnosed patients with pemphigus vulgaris. Methods: In this study, 50 newly diagnosed patients with pemphigus vulgaris admitted to Razi Hospital in Tehran were studied with regards to their serum levels of copper and zinc alongside age, sex, body mass index (BMI), smoking and drug abuse status. Results: Eighteen percent (9 cases) and 50% (25 cases) of the patients showed copper and zinc deficiency, respectively. There was no significant association between copper and zinc levels and smoking, age, BMI or suffering from a chronic disease (P>0.05). Mean copper levels in women and men were not significantly with their respective different normal levels (P>0.05). Serum zinc level in men was lower than its normal range (P<0.05), but in women was not significantly different from its normal level. Conclusion: Serum concentration of copper and zinc did not show any association with pemphigus disease. Since decreased levels of copper and zinc may result in an increase of oxidative activity in autoimmune diseases enhancing disease severity, therefore conducting a robust case-control study for evaluating the their roles in disease severity may pave the way to treatment of those diseases.

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Background and Aim: Pemphigus vulgaris is the most common autoimmune blistering disease. Prednisone, commonly in combination with an immunosuppressive adjuvant such as azathioprine or cyclophosphamide, is the standard treatment for pemphigus vulgaris. Approximately 5% to 15% of mortality of the disease is due to the complications of corticosteroids therapy. The aim of this study was to determine the prevalence and risk factors of steroid-induced diabetes in pemphigus patients hospitalized in Razi hospital, Tehran in 2009 and 2010.

Methods: In this cross-sectional study, 177 first-time admitted pemphigus vulgaris patients were studied regarding presence of risk factors for steroid-induced diabetes. Those risk factors were included age, sex, blood pressure, body mass index (BMI), family history of diabetes, medications used before and after initiation of treatment, fasting blood sugar (FBS) , triglyceride (TG) high density lipoprotein (HDL), low density lipoprotein (LDL) levels at the time of admission, and three weeks after the treatment was started.

Results: Twenty-nine patients (16.3%) were diagnosed with diabetes based on three weeks FBS levels evaluations. There were significant associations between BMI, HbA1c and taking nervous system agents and diabetes (P<0.05), but the differences between sex, family history, blood pressure, TG, LDL levels and occurrence of diabetes were not significant (P>0.05).

Conclusion: Close monitoring of FBS levels before and within the first three weeks of the initiation of steroid therapy allows early detection of storied-induced diabetes in pemphigus patients. Moreover, evaluation of other risk factors associated with diabetes may provide the opportunity of early diagnosis and treatment of steroid-induced diabetes.

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Background and Aim: Pemphigus is a chronic autoimmune blistering disease characterized by intraepidermal blisters.
These blisters damage the skin barrier and increase the risk of life-threatening infections. Nasal colonization of
Staphylococcus aureus can increase the risk of infections and delay the wound healing process.The aim of this study was to
determine the frequency of staphylococcal carriers in hospitalized patients with pemphigus.
Methods: In this prospective cross-sectional study, medical records of 72 hospitalized patients with pemphigus in the
period from March 2013 to March 2014 were reviewed.Samples were collected from anterior nares and cultured at the
time of admission and one week after hospitalization for evaluation of S. aureus colonization and Methicillin-resistant
Staphylococcus aureus (MRSA) types.
Results: Forty-five patients (62.5%) were male and 27 patients (37.5%) were female. The frequency of S. aureus
carriers was 58.3%. Thirty patients had colonization at the time of hospital admission among them, 22 (73.3%) patients
had MRSA. In forty (55.6%) patients colonization were found one week after hospitalization. Thirty-three (82.5%) patients
in this group were MRSA positive. There was no significant difference between S. aureus colonization at the time of
admission and one week after hospitalization (P>0.05).
Conclusion: S. aureus and MRSA carriers (communityacquired and hospital-acquired carriers) were prevalent
among patients with pemphigus. With identifiction and isolation of carriers of S. aureus in pemphigus patients, not
only could prevent spreading S. aureus and MRSA associated infections, bul also would facilitate wound healing. More
robust studies are required to assess the role of potential riskfactors.