Nikoo Mozafari; Fariba Ghalamkarpour; Zohreh Rakhshan
Abstract
Background: Pemphigus vulgaris (PV) is a rare autoimmune disease characterized by the development of flaccid blisters on the skin and mucous membranes. Detection of anti-desmoglein (Dsg) 1 and anti- Dsg3 antibodies are frequently used for diagnosing the disease and evaluating disease activity. Recently, ...
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Background: Pemphigus vulgaris (PV) is a rare autoimmune disease characterized by the development of flaccid blisters on the skin and mucous membranes. Detection of anti-desmoglein (Dsg) 1 and anti- Dsg3 antibodies are frequently used for diagnosing the disease and evaluating disease activity. Recently, the neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), and mean platelet volume (MPV) were introduced as new biomarkers indicating inflammation in autoimmune and autoinflammatory diseases. We aimed to evaluate the possible associations of NLP, PLR, and MPV with pemphigus disease severity and anti-Dsg1/3 levels.Methods: Thirty-three newly diagnosed cases of PV and 33 age and sex-matched controls were included in this study. A complete blood count (CBC) was obtained from the participants to evaluate NLP, PLR, and MPV. Serological anti-Dsg1/3 and Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) were assessed in patients based on ELISA assay and clinical examination, respectively.Results: The median (interquartile range) NLR and PLR values in patients were 2.50 (1.94–6.59) and 90.30 (71.60–196.80), respectively, compared with 1.69 (1.45–2.30) and 56.00 (50.00–85.00) in controls. The NLR and PLR were significantly higher in patients than in controls (P < 0.001 for both). However, no significant difference regarding MPV levels was detected. Neither the ABSIS nor the anti- Dsg1/3 levels correlated with the studied inflammatory markers.Conclusion: Our study revealed that NLR and PLR are elevated in patients with PV but do not correlate with disease activity (evaluated by the ABSIS) or anti-Dsg1/3 levels. These laboratory parameters can be considered inflammatory markers of PV but cannot predict the disease activity.
Emadodin Darchini-Maragheh; Sara Fakhraei; Pouran Layegh
Abstract
Multiple endocrine neoplasia type 1 (MEN1) is a rare, autosomal dominant hereditary syndrome caused by mutations of the MEN1 tumor suppressor gene. We describe a patient with a prior confirmed diagnosis of pemphigus vulgaris (PV), who presented with new cutaneous manifestations that led to the diagnosis ...
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Multiple endocrine neoplasia type 1 (MEN1) is a rare, autosomal dominant hereditary syndrome caused by mutations of the MEN1 tumor suppressor gene. We describe a patient with a prior confirmed diagnosis of pemphigus vulgaris (PV), who presented with new cutaneous manifestations that led to the diagnosis of MEN1. A man in his early forties with a history of PV from 11 years ago presented with some cutaneous lesions six months ago, diagnosed as angiofibroma and collagenoma. Moreover, he suffered from recurrent renal stones and gout for several years. Laboratory analysis showed hypercalcemia, which led us to confirm the diagnosis of a parathyroid adenoma by sestamibi scintigraphy. Contrast-enhanced computed tomography (CT) of the abdomen revealed hyper-enhancing pancreatic lesions while the patient had no related symptoms. Thus, the clinical diagnosis of MEN1 syndrome was settled, and the patient underwent surgical and medical management. A hitherto unreported cooccurrence between MEN-1, as a hereditary syndrome, and PV, as an autoimmune bullous cutaneous dermatosis, opens a hazy challenging issue: whether MEN-1 has any association with autoimmune bullous cutaneous diseases like PV or increases the incidence of such conditions.
Akram Momenzadeh; Mahboubeh Khajavi; Mohammad Reza Kazemi; Mehrdad Teimoorian; Fatemeh Livani; Masoud Saleh Moghadam; Pouran Layegh
Abstract
Background: Pemphigus vulgaris (PV) is an autoimmune disease with worldwide distribution. Human leukocyte antigen G (HLA-G) is postulated to be associated with this inflammatory and autoimmune condition. However, its role has not been well established in the literature. The study aimed to evaluate the ...
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Background: Pemphigus vulgaris (PV) is an autoimmune disease with worldwide distribution. Human leukocyte antigen G (HLA-G) is postulated to be associated with this inflammatory and autoimmune condition. However, its role has not been well established in the literature. The study aimed to evaluate the plasma level of HLA-G in PV patients and assess its correlation with disease severity and compare it with normal subjects.Methods: Thirty PV patients were enrolled in this cross-sectional study. A blood sample was taken from each participant; samples were analyzed for the soluble HLA-G (sHLA-G) plasma level by applying an ELISA kit (sHLA-G ELISA kit; Exbio, Czech Republic). Patients’ clinical and demographic data were recorded and analyzed.Results: Higher levels of sHLA-G were seen in PV patients compared to the control group (P < 0.05). There was a negative linear relationship between plasma HLA-G level and PV based on all ABSIS indices except for oral involvement (-1 < R < 0); however, these correlations were not statistically significant (P≥0.05).Conclusion: Our data showed higher plasma sHLA-G levels in PV patients, which did not correlate with disease severity.
Maryam Zahed; Ladan Dastgheib; Maryam Sadat Sadati; Asma Soukhakian
Abstract
Background: Pemphigus vulgaris (PV) is a life-threatening autoimmune disease that affects the skin and mucosa. In most regions, little data is available on its epidemiology. This study aimed to present an overview of the worldwide epidemiology of pemphigus and evaluate the epidemiological features of ...
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Background: Pemphigus vulgaris (PV) is a life-threatening autoimmune disease that affects the skin and mucosa. In most regions, little data is available on its epidemiology. This study aimed to present an overview of the worldwide epidemiology of pemphigus and evaluate the epidemiological features of PV in the southwest of Iran.Methods: In this study, data including sex, age, time of disease onset, phenotype of the disease, geographic location of birth, geographic location of residence, level of education, and occupation of PV patients from 2004 to 2019 were collected from the records existing in the Oral Medicine Department of Shiraz Dental School and Shahid Faghihi Hospital, Shiraz, Iran.Results: In total, 249 PV patients with a mean age of 42.72 ± 13.3 years were included. The female to male ratio was 1.74:1. The annual incidence of PV in Fars province was 2.7 per million people. In this region, the most frequent phenotype was the mucocutaneous phenotype in 204 patients (81.9%), followed by the mucosal dominant phenotype in 40 (16.1%) and the cutaneous phenotype in 5 (2%). Regarding the level of education, 43.4% had less than a high school diploma, 24.9% had a high school diploma, 17.3% had an associate diploma or higher, and 14.5 % were illiterate. Moreover, 55.8% were housewives, 21.7% were self-employed, 19.3 % were employed, and 3.2% were jobless.Conclusion: The findings indicate that PV is a rare disease in the southwest of Iran compared with other regions. According to previous studies in this region, the incidence has decreased since 2005.
Banihashemi Mahnaz; Nahidi Yalda; Meibodi Naser Tayyebi; Jarrahi Lida; Livani Fatemeh; Seifnia Sahar
Abstract
Background: Pemphigus vulgaris (PV) is a rare autoimmune blistering disease of the skin and mucous membranes. Because vitamin D deficiency is associated with many immune disorders, we compared the levels of vitamin D between PV patients and healthy controls. Methods: Vitamin D level of 20 patients with ...
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Background: Pemphigus vulgaris (PV) is a rare autoimmune blistering disease of the skin and mucous membranes. Because vitamin D deficiency is associated with many immune disorders, we compared the levels of vitamin D between PV patients and healthy controls. Methods: Vitamin D level of 20 patients with PV was compared with 20 healthy individuals matched for age, gender, hours spent in the sun, body mass index (BMI) and daily vitamin D intake at the same time interval. The severity of the disease was estimated according to ABSIS score. The serum level of 25-hydroxyvitamin D (25OHD) was measured by a commercial ELISA test. Results: Vitamin D deficiency in the pemphigus vulgaris group (75%) was higher than healthy controls (45%) (P = 0.053). Mean level of vitamin D in PV patients was significantly lower than healthy controls, and was in the range of vitamin deficiency (P ? 0.05). No significant relationship was observed between the severity of the PV disease and vitamin D levels (P > 0.05). Conclusions: Based on the results of the present study, the mean level of vitamin D in patients with PV was significantly lower than healthy people. Based on our findings and given the important role of vitamin D in preventing inflammatory and autoimmune diseases, vitamin D deficiency can be considered as a factor triggering the onset of PV.
Shahidi-Dadras Mohammad; Kheradmand Zohreh; Abdollahimajd Fahimeh
Abstract
Background: Recently, rituximab has been successfully used for the treatment of pemphigus family, the main subtype of which is pemphigus vulgaris (PV). The aim of this study was to determine the efficacy and safety of rituximab in refractory PV. Methods: In an observational study extending from November ...
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Background: Recently, rituximab has been successfully used for the treatment of pemphigus family, the main subtype of which is pemphigus vulgaris (PV). The aim of this study was to determine the efficacy and safety of rituximab in refractory PV. Methods: In an observational study extending from November 2014 to February 2016, 30 patients with refractory PV were treated with rituximab. Response to therapy, duration of clinical remission, relapse rate, serology, and side effects of treatment with rituximab were evaluated. Results: At the end of the follow-up with a mean duration of 7.6 (6-14) months, 25 (83.3%) patients achieved complete remission with or without systemic therapy. The mean duration of disease control was 2.8 months. In all patients, the serum levels of antidesmoglein 1 and 3 IgG antibodies were reduced after rituximab therapy. In 23.4% of the patients, a clinical relapse occurred at a mean of 9.6 months following the initiation of the treatment. Infusion-related reactions occurred in 18 (60%) patients. The lack of a control group, concomitant use of corticosteroid and immunosuppressive agents, and a limited follow-up period were among the limitations of our research. Conclusions: Rituximab is a good treatment modality for refractory PV, which extends the mean time to relapse in patients. To further extend our knowledge on the efficacy and safety of rituximab therapy, more randomized controlled trials with larger sample sizes and prolonged follow-up durations are required.
Farhad Handjani; Nasrin Saki; Motahareh Hosseini; Taraneh Tadayon
Volume 20, Issue 3 , 2017, , Pages 84-88
Abstract
Background: Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) are 2 inflammatory indicators that increase in many pathologic and physiologic conditions. Pemphigus vulgaris (PV) is an autoimmune skin disease in which autoantibodies against desmogleins cause acantholysis. In this study ...
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Background: Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) are 2 inflammatory indicators that increase in many pathologic and physiologic conditions. Pemphigus vulgaris (PV) is an autoimmune skin disease in which autoantibodies against desmogleins cause acantholysis. In this study we have measured ESR and CRP levels in patients with PV to evaluate the relationship between an increase in these markers and disease severity.Methods: In this cross-sectional study, we selected patients with proven diagnosis of PV who referred to the Dermatology referral clinic, Faghihi hospital, Shiraz, Iran during a one year period. There were 30 patients enrolled in this study who provided blood samples to measure their ESR and CRP levels. We usedthe Mahajan scoring system to determine the disease severity. Results: In 27 (90%) cases, ESR was normal and 20 (66.7%) cases had negative CRP. There was a significant correlation between CRP and PV severity (P=0.015); however, there was no correlation between ESR and disease severity (P=0.126). Conclusion: A correlation existed between CRP and severity of PV. Further studies must be undertaken to prove the prognostic role of CRP in PV. The discovery of new prognostic factors can change the treatment strategy and protocol for PV.
Ghiasi Maryam; Daneshpazhooh Maryam; Balighi Kamran; Ghiasi Fatemeh
Volume 20, Issue 1 , 2017, , Pages 6-10
Abstract
Background: Intravenous immunoglobulin (IVIG) is used to treat many autoimmune and immunodeficiency disorders. The main indications of IVIG in dermatology include treatment for resistant autoimmune bullous diseases, Stevens-Johnson syndrome (SJS), and toxic epidermal necrolysis (TEN). Although generally ...
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Background: Intravenous immunoglobulin (IVIG) is used to treat many autoimmune and immunodeficiency disorders. The main indications of IVIG in dermatology include treatment for resistant autoimmune bullous diseases, Stevens-Johnson syndrome (SJS), and toxic epidermal necrolysis (TEN). Although generally welltolerated, various adverse effects can occur.Methods: We designed a retrospective study to investigate the adverse effects of IVIG in all patients who received this drug in Razi Hospital from 2005-2016. Information was gathered from patients’ medical records.Results: During the study period, 67 patients received 94 IVIG infusions. The most common underlying dermatologic disease was pemphigus vulgaris (54 patients). The most frequent adverse effect of IVIG therapy was an increase in blood pressure for 17 patients and in 21 infusions of IVIG. Other adverse reactions included fatigue and generalized weakness, fever, chills, tachycardia, dizziness, a decrease in blood pressure, headache, flushing, chest discomfort, hemolysis, leukopenia, and deep vein thrombosis.Conclusions: Adverse events associated with IVIG therapy are usually mild and self-limiting. The incidence of serious adverse events is low. Identification of risk factors and close monitoring of high risk patients are essential to decrease the occurrence of serious adverse events.
Narges Ghandi; Shaghayegh Tavassoli; Maryam Ghiasi; Vahideh Lajevardi; Robabeh Abedini; Hamid-Reza Tohidinik; Maryam Daneshpazhooh
Volume 19, Issue 2 , 2016, , Pages 35-39
Ghandi Narges; Tavassoli Shaghayegh; Ghiasi Maryam; Lajevardi Vahideh; Abedini Robabeh; Tohidinik Hamid-Reza; Daneshpazhooh Maryam
Volume 19, Issue 75 , 2016, , Pages 35-13
Abstract
Background: Prolactin (PRL) appears to play a role in the pathogenesis of autoimmune diseases. Limited evidence showed an association between serum PRL levels and the activity of pemphigus vulgaris (PV). This study intends to determine PRL level changes in pemphigus patients during therapy and its correlation ...
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Background: Prolactin (PRL) appears to play a role in the pathogenesis of autoimmune diseases. Limited evidence showed an association between serum PRL levels and the activity of pemphigus vulgaris (PV). This study intends to determine PRL level changes in pemphigus patients during therapy and its correlation with disease type and severity. Methods: In this cohort study, we measured serum PRL levels by enzyme-linked immunosorbent assay (ELISA) in newly diagnosed PV patients at three time points: before therapy initiation and after two and four months. Concomitantly, we estimated disease severity by the Pemphigus Disease Area Index (PDAI). Results: We examined 42 new cases of PV. Among 32 cases who completed the study, mean serum PRL levels at the three time points were 15.9±14.1 ng/mL (before treatment), 16.7±9.8 ng/ mL (2 months after initiation of treatment), and 15.2±9.2 ng/mL (4 months after initiation of treatment). Mean PDAI values were 19.3±12.8 (before treatment), 3.7±6.2 (2 months after initiation of treatment), and 0.6±1.5 (4 months after initiation of treatment). Although the disease activity decreased significantly (P
Taheri Ahmadreza; Tanipour Mohammad Hossein; Khorasani Zahra Kafami; Kiafar Bita; Layegh Pouran; Hashemy Seyed Isaac
Volume 18, Issue 4 , 2015, , Pages 156-162
Abstract
Background: Pemphigus diseases including pemphigus vulgaris (PV) and bullous pemphigoid (BP) are autoimmune diseases that cause severe blistering of the skin and mucous membranes. Among inflammatory mediators, reactive oxygen species (ROS) are involved in the pathogenesis of a wide variety of diseases ...
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Background: Pemphigus diseases including pemphigus vulgaris (PV) and bullous pemphigoid (BP) are autoimmune diseases that cause severe blistering of the skin and mucous membranes. Among inflammatory mediators, reactive oxygen species (ROS) are involved in the pathogenesis of a wide variety of diseases through oxidative stress for which protein carbonyl (PC) and total antioxidant capacity (TAC) are two important markers.Methods: We utilized PC and TAC in this study to compare the serum redox status of PV and BP patients with healthy subjects to investigate the possible ro
Dadras Mohammad Shahidi; Golfeshan Atefeh; Younespour Shima
Volume 18, Issue 1 , 2015, , Pages 16-19
Abstract
Background: The ABO blood group antigens play a role in thepathophysiology of some diseases and several researches haveinvestigated this relationship in the field of dermatology. In thepresent study, an attempt was made to find the distribution ofblood types in patients with pemphigus vulgaris and chronicplaque ...
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Background: The ABO blood group antigens play a role in thepathophysiology of some diseases and several researches haveinvestigated this relationship in the field of dermatology. In thepresent study, an attempt was made to find the distribution ofblood types in patients with pemphigus vulgaris and chronicplaque type psoriasis.Method: Fifty patients with chronic plaque type psoriasis, 50patients with pemphigus vulgaris and 100 healthy persons ascontrol group, were selected as the study population. ABO bloodgrouping was determined by the cell type tube test method, usingstandard commercial anti/A, anti/B and 5% suspension of redblood cells. Chi square test was used to compare the frequencyof ABO blood groups in patients and control groups.Result: Among the 50 patients with psoriasis, 21 (42%) hadblood group O, 15 (30%) blood group A, 10 (20%) blood groupB and 8 (4%) had blood group AB. Among the 50 patients in thepemphigus group, 22 (44%) had blood group O, 19 (38%) bloodgroup A, 8 (16%) blood group B and 1 (2%) had blood groupAB. The distribution of blood groups in patients with these twodiseases was not significantly different from the control group.Conclusion: Despite some researches, which found associationsbetween blood group antigens and psoriasis or pemphigus, thisstudy did not find any such relationship.
Layegh Pouran; Nahidi Yalda; Malekzadeh Iman; Shakeri Mohammad Taghi
Volume 16, Issue 3 , 2013, , Pages 100-104
Abstract
Background: Pemphigus is a rare autoimmune disease causedby autoantibodies against desmoglein. It clinically presents withpainful blisters and erosions on the skin and mucous membranes.Few studies have been conducted on the quality of life inpemphigus patients which have all indicated the strong impactof ...
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Background: Pemphigus is a rare autoimmune disease causedby autoantibodies against desmoglein. It clinically presents withpainful blisters and erosions on the skin and mucous membranes.Few studies have been conducted on the quality of life inpemphigus patients which have all indicated the strong impactof the disease on emotional and physical status of the patients.According to evident differences in the culture and quality oflife between our society and western societies, we assessed thequality of life in patients with pemphigus in the Northeast of Iran.Method: This study was conducted on 78 pemphigus vulgarispatients who were visited or hospitalized in the dermatologyclinics of Qaem and Imam Reza Hospitals in Mashhad, Iran.Data collection was done by the Dermatology Life Quality Index(DLQI) questionnaire in patients with pemphigus vulgaris.Statistical analysis was performed using SPSS 11.5 software withChi-square, T-test and ANOVA statistical tests.Result: The disease had a significant impact on the quality oflife in the majority of patients (31 (39.7%) patients). Hospitalizedand newly diagnosed cases and those receiving higher doses ofsteroid had lower quality of life. The patient’s age, sex, educationlevel, and therapeutic regimen did not have a significant effecton the quality of life.Conclusion: Pemphigus is not just a physical disease and hasvarious aspects. It is responsible for many changes in healthrelatedquality of life in patients. Therefore, we will be successfulin treating this disease only when psychological and social aspectsof pemphigus are considered in addition to clinical improvementof the patients.
Parviz Toossi; Somayeh Hejazi; Safoura Shakoei; Zahra Asadi-Kani
Volume 16, Issue 3 , 2013, , Pages 109-111
Abstract
Pemphigus vulgaris (PV) is a potentially fatal autoimmune mucocutaneous blistering disease. Although PV occurs predominantly in adulthood, in the 3rd to 5th decades of life, there have rarely been reports of childhood cases which are often misdiagnosed. It presents as oral blisters ...
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Pemphigus vulgaris (PV) is a potentially fatal autoimmune mucocutaneous blistering disease. Although PV occurs predominantly in adulthood, in the 3rd to 5th decades of life, there have rarely been reports of childhood cases which are often misdiagnosed. It presents as oral blisters that rupture rapidly and progress to painful erosions. Most patients develop cutaneous flaccid blisters that rupture easily and leave painful erosions, which are slow to heal. These erosions are prone to secondary bacterial infection. Without treatment, the disease is progressive and the mortality rate is about 100%. We report an 8-year-old girl who was referred to our clinic from a pediatric center with mucocutaneous recalcitrant blisters.
Esmaili Nafiseh; Chams-Davatchi Cheyda; Daneshpazhooh Maryam; Ghiasi Maryam; Abedini Robabe; Mortazavi Hossein; Roghani Iman
Volume 15, Issue 2 , 2012, , Pages 33-37
Abstract
Background: Pemphigus vulgaris (PV) is an autoimmune bullous disorder that is fatal if left untreated. High dose systemic corticosteroids are the basis of therapy. The addition of immunosuppressive agents has improved the disease outcome and reduced the required corticosteroid dose and related toxicity. ...
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Background: Pemphigus vulgaris (PV) is an autoimmune bullous disorder that is fatal if left untreated. High dose systemic corticosteroids are the basis of therapy. The addition of immunosuppressive agents has improved the disease outcome and reduced the required corticosteroid dose and related toxicity. Mycophenolate mofetil is increasingly used as a steroid-sparing agent in immunotherapy of PV. Herein, we tried to appraise the efficacy of mycophenolate mofetil and topical clobetasol in the control of the major relapses of pemphigus vulgaris. Method: Seventeen patients with severe relapse of pemphigus vulgaris were included in this study. All patients had complete remission on/off therapy before this period of recurrence. The patients were treated with 2g/day mycophenolate mofetil and 25-35g/day topical clobetasol propionate ointment. All patients were monitored for the side effects of therapy. Result: The patients were followed for a mean period of 12.7 months. The average length of time from initiating mycophenolate to 50% control (partial remission), which occurred in all patients, was 6±1.17 weeks. Fifteen patients achieved complete remission averagely at week 20.8±7.70. The average duration of followup after complete disease control was 8 months (ranging from 2-13.5 months). Three patients were free of lesions for more than 12 months and 10 for more than 6 months. No important mycophenolate mofetil related complication was observed during treatment. Conclusion: The combination of mycophenolate mofetil and topical corticosteroid can be used to control PV relapses and taper-off corticosteroid.
Niloufar Torkamani; Eskandar Kamali Sarvestani; Mahsa Naseri
Volume 14, Issue 1 , 2011, , Pages 16-19
Abstract
Background: Pemphigus vulgaris, the most common form of pemphigus, is due to the production of auto-antibodies directed against adhesion molecules (desmoglein 1 and 3) that belong to the cadherin family. B cell-activating factor of the TNF family (BAFF) and a proliferation-inducing ligand (APRIL) may ...
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Background: Pemphigus vulgaris, the most common form of pemphigus, is due to the production of auto-antibodies directed against adhesion molecules (desmoglein 1 and 3) that belong to the cadherin family. B cell-activating factor of the TNF family (BAFF) and a proliferation-inducing ligand (APRIL) may play a significant role in autoimmune diseases such as pemphigus vulgaris. Only a few studies have been done on the level of APRIL and BAFF in PV patients but none have investigated both levels; therefore, determination of both proliferation inducing ligands (BAFF and APRIL) in pemphigus vulgaris is of significant importance and was considered as the goal of the present study. Methods: This analysis included 22 patients with PV and 22 sex and age matched healthy controls. None of the patients had previously been treated with corticosteroids or any immunosuppressive drugs in the previous three months. The BAFF and APRIL levels were evaluated in patient and control groups by use of ELISA method. Results: Comparison of the serum levels of BAFF and APRIL between patients and controls was done using Mann-Whitney U test. The BAFF levels in our 22 patients and control sera were undetectable. On the other hand, concentrations of APRIL in 22 cases were 2.09+/-4.94 and 0.85+/-2.01 in the control group. Conclusion: BAFF levels were undetectable in cases and the difference in APRIL levels between patient and control groups was not significant (p=0.28). So, it can be concluded that although BAFF and APRIL may play a role in autoimmune diseases, their role in pemphigus vulgaris is doubtable.
Nafiseh Esmaily; Amirhooshang Ehsani; Pedram Noormohammadpour; Iman Baiagouy; Shima Sayanjali
Volume 13, Issue 4 , 2010, , Pages 118-121
Abstract
Background: Pemphigus vulgaris (PV) is a rare autoimmune blistering disease with a high rate of morbidity and mortality without treatment. Until now, treatments consist mostly of the use of corticosteroid and immunosuppressive drugs. Optimum dosage for corticosteroid therapy is yet to be determined. ...
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Background: Pemphigus vulgaris (PV) is a rare autoimmune blistering disease with a high rate of morbidity and mortality without treatment. Until now, treatments consist mostly of the use of corticosteroid and immunosuppressive drugs. Optimum dosage for corticosteroid therapy is yet to be determined. The aim of the present study was to clear whether different initial corticosteroid therapeutic dosages change the recurrence rate of the disease.Methods: Patients hospitalized with confirmed PV from 2000 to 2006 were enrolled if they received systemic corticosteroids without any adjuvant therapy for no more than three weeks when hospitalized, received their first treatment in the period of the study and were followed for at least two years in the PV clinic of Razi Hospital periodically. Initial steroid dosage as well as the number and type of the recurrence(s) were documented and analyzed.Results: A total of 62 patients with a mean age of 46.8 (± 15.9) were enrolled. Twenty-one patients were female (33.9%) and 41 patients (66.1%) were male. According to the initial dose of systemic corticosteroid, patients were divided into two groups: group A included patients treated with less than 2mg/kg (10 patients or 16.1%) while group B included patients treated with 2mg/kg systemic steroids (52 patients or 83.9%). There was no significant difference in the recurrence rate between the two groups. Recurrence rate was 40% in group A and 51% in group B (P>0.05). According to the type of recurrence, there was a significant difference between the two groups; in group A, the first recurrence was major in 33% of the patients while in group B, this rate was about 28.8% (P<0.05).Conclusion: According to the present study, initial therapeutic dosage did not influence the recurrence rate although the type of recurrence was influenced. Patients treated with higher initial steroid dosages experienced their first episode as a minor recurrence while patients treated with lower initial steroid dosages experienced major recurrences with a higher probability as their first episode.
Shahidi-Dadras Mohammad; Farnaghi Ali; Tehranchinia Zohreh; Rahimi Hoda; Saeedi Marjan; Ghaemi Marjan
Volume 13, Issue 3 , 2010, , Pages 67-70
Abstract
Introduction: Pemphigus vulgaris is an autoimmune blistering disease of the skin and mucous membrane. Antiphospholipid antibodies are a group of autoantibodies found in patients with autoimmune diseases. Due to the risk for thrombotic events in autoimmune diseases, we decided to evaluate the correlation ...
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Introduction: Pemphigus vulgaris is an autoimmune blistering disease of the skin and mucous membrane. Antiphospholipid antibodies are a group of autoantibodies found in patients with autoimmune diseases. Due to the risk for thrombotic events in autoimmune diseases, we decided to evaluate the correlation between pemphigus vulgaris and predictive elements of atherothrombosis including antiphospholipid antibodies and homocysteine. Methods: IgG and IgM anticardiolipin antibodies, IgG anticardiolipinbeta 2 glycoprotein I, lupus anticoagulant and total homocysteine were evaluated in the serum of 39 new cases of pemphigus vulgaris and healthy matched controls. Results: The prevalence of antiphospholipid antibodies, lupus anticoagulant and total homocysteine in pemphigus vulgaris patients showed no significant difference with healthy controls. Conclusion: Pemphigus vulgaris seems not to be similar to some other organ specific autoimmune diseases in which there is an increase in atherothrombotic factors including antiphospholipid antibodies and homocysteine.
Vora Deval; Popat Vijay; Bhanvadia Viral; Mehta Dimple A.; Bhetariya Bharat; Kumar Meet
Volume 13, Issue 2 , 2010, , Pages 42-46
Abstract
Background: A clinicopathological study of 75 cases of pemphigus was carried out at Saurashtra region of Gujarat State, India. This study was done to subtype this disease with evaluation of clinical and histopathological presentation. Methods: The study was carried out in a two-year period at skin department ...
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Background: A clinicopathological study of 75 cases of pemphigus was carried out at Saurashtra region of Gujarat State, India. This study was done to subtype this disease with evaluation of clinical and histopathological presentation. Methods: The study was carried out in a two-year period at skin department of Guru Gobind Singh Hospital, Jamnagar. Seventy five patients of pemphigus were examined, admitted and treated. Histopathological examination was done at the department of pathology and direct immunoflouroscence was advised and done at higher centers when it was needed. Result: Out of 75 cases, pemphigus vulgaris constituted the single largest group of cases which made 72 (96%) cases with three (4%) cases of pemphigus foliaceus. The majority of the cases were seen in the age group of 21-60 years, with a slight female predominance. The youngest patient was 18 years while the eldest was 70 years old. Oral mucosal involvement was seen in almost all cases of pemphigus vulgaris while 7 cases of pemphigus vulgaris had mucosal involvement at other sites in addition to oral mucosal involvement. No mucosal involvement was present in cases of pemphigus foliaceus. Flaccid bullae were present in 100% of the cases. Pruritus was present in 15 cases of pemphigus vulgaris. Conclusion: Most of the patients had pemphigus vulgaris followed by pemphigus foliaceus. Pemphigus is more prevalent at certain areas of Saurashtra region showing a geographic distribution.
Mortazavi Hossein; Amirzargar Ali Akbar; Valikhani Mahin; Hallaji Zahra; Daneshpazhouh Maryam; Tabrizi Mohammad Javad Nazemi; Seirafi Hassan; Nikbin Behrouz; Khosravi Farideh; Toosi Siyavash; Chams-Davatchi Cheida
Volume 11, Issue 1 , 2008, , Pages 11-16
Abstract
Background: In addition to humoral immunity associated with anti-desmoglein antibodies, cellular immunity and mediators including cytokines are involved in the pathogenesis of pemphigus vulgaris. In this study we evaluated the level of IL-2, IFN-γ, TNF-α, IL-4, and IL-10 in the sera of patients ...
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Background: In addition to humoral immunity associated with anti-desmoglein antibodies, cellular immunity and mediators including cytokines are involved in the pathogenesis of pemphigus vulgaris. In this study we evaluated the level of IL-2, IFN-γ, TNF-α, IL-4, and IL-10 in the sera of patients with pemphigus vulgaris before and after treatment.Methods: A total number of 71 new patients with pemphigus vulgaris were included in the study. The above mentioned cytokines were measured in patients with a mild disease (20 bullae or less) and a severe disease (60 bullae or more) using ELISA method before and 4 weeks after treatment with 2 mg/kg/day prednisolone and 2mg/kg/day azathioprine. We also measured IL-4 and IL-10 in 69 mildly and 70 severely affected patients only at the beginning of the study. All patients had muco-cutaneous phenotype. Patients with a mild disease had mild mucosal involvement and patients with a severe disease had moderate to severe mucosal involvement. Serum levels of IL-2 and IFN-γ were also measured in 27 normal controls.Results: In the total study population, the level of IL-2 decreased from 103.9 pg/ml to 82.79 pg/ml after treatment (p=0.05). Comparing cytokines between 2 groups (severe and mild), the level of IL-2 before treatment showed significantly lower figures in severe patients (147.27 versus 67.38, p=0.04). On the other hand, IFN-γ after treatment was significantly higher in severe patients (0.75 versus 0.42, p=0.04).Conclusion: Mean level of IL-2 is lower in severe pemphigus vulgaris patients than that of mild disease. This finding indicates that, in pemphigus vulgaris, IL-2 level negatively correlates with the severity of the disease and widespread underlying autoimmune process. The data also suggests that the level of IFN-γ directly correlates with the severity of the disease.
Maryam Akhyani; Cheyda Chams-Davatchi; Zahra Safai Naraghi; Maryam Daneshpazhooh; Siavash Toosi; Masoud Asgari; Fatemeh Malek Hami
Volume 10, Issue 3 , 2007, , Pages 194-202
Abstract
Background and aim: Pemphigus vulgaris (PV) is an autoimmune blistering disease which is known to involve the female genital tract but the frequency at which this occurs is unknown. There are relatively few reports in the literature of the cytological appearance of PV on cervicovaginal smears. Our aim ...
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Background and aim: Pemphigus vulgaris (PV) is an autoimmune blistering disease which is known to involve the female genital tract but the frequency at which this occurs is unknown. There are relatively few reports in the literature of the cytological appearance of PV on cervicovaginal smears. Our aim was to evaluate involvement of the female genital tract and cervicovaginal Papanicolaou (Pap) smears in PV.Materials and methods: The study included 77 patients with PV visited from April 2005 to February 2007. Each patient was subjected to gynecological examination and cervicovaginal Pap smear.Results: The mean age of patients was 44.7±13 years. Genital lesions were observed in 39 patients (50.6%). Labia minor was involved in 36 patients (92.3%), while labia major in 11 (28.2%), vagina in 14 (35.8%), and cervix in six (15.3%). Cervicovaginal smears of 20 patients (25.9%) showed PV. Among 72 satisfactory smears, cervical Pap smears were normal in 25 patients (34.7%), inflammatory in 43 patients (59.7%), and dysplastic (low-grade cervical intra-epithelial neoplasia [CIN I]) in four patients (5.5%). In 2 of these patients, Pap smears was normal repeated when patients were in remission.Conclusion: Involvement of the female genital tract might not be as infrequent as was previously thought and it is probably the second most common mucosal site of PV after oral mucosa. Genital lesions may be missed and the need for thorough pelvic examination should not be overlooked.
Chams-Davatchi Cheyda; Balighi Kamran; Yazdanian Shideh; Nabaii Behruz
Volume 10, Issue 2 , 2007, , Pages 111-118
Abstract
Background and aim: Pemphigus vulgaris is an autoimmune vesiculobullous disorder. Prior to the advent of glucocorticoid therapy, it was often fatal. Now its mortality and morbidity is due to highdose glucocorticoid complications rather than the disease itself. This study was aimed to determine the effect ...
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Background and aim: Pemphigus vulgaris is an autoimmune vesiculobullous disorder. Prior to the advent of glucocorticoid therapy, it was often fatal. Now its mortality and morbidity is due to highdose glucocorticoid complications rather than the disease itself. This study was aimed to determine the effect of dapsone as a second adjuvant in the treatment of pemphigus vulgaris.Materials and methods: In this quasi-experimental open prospective study, 22 patients with moderate to severe pemphigus vulgaris were treated with prednisolone, azathioprine and dapsone. They were observed for 12-18 months.Results: Patients consisted of 10 females and 12 males with mean age of 41.71 years. The mean duration to reach to the maintenance dose of prednisolone (7.5 mg daily) was 13 months. No major relapse was observed but minor relapses were seen in 6 patients with mean prednisolone dose of 13.8 mg daily. Hemolysis was detected in only one patient and 4 patients had a little drop in hemoglobin level. We observed no other severe dapsone induced adverse reaction.Conclusion: These findings suggest that dapsone can be used as a second adjuvant for more rapid tapering of steroid dosage without significant flare of the disease.
K Balighi; Sh Yazdanian; N Ghandi
Volume 9, Issue 3 , 2006, , Pages 284-288
Abstract
Pemphigus vulgaris is a blistering autoimmune disease with suprabasal cleft formation, which is the most common autoimmune blistering disease in eastern countries, such as Middle East. Predisposition to pemphigus is linked to genetic factors. Tuberous sclerosis is also a genetic disorder of hamartoma ...
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Pemphigus vulgaris is a blistering autoimmune disease with suprabasal cleft formation, which is the most common autoimmune blistering disease in eastern countries, such as Middle East. Predisposition to pemphigus is linked to genetic factors. Tuberous sclerosis is also a genetic disorder of hamartoma formation in many organs, particularly the skin, brain, eye, kidney and heart. We report a 17-year-old boy with pemphigus vulgaris associated with tuberous sclerosis, which to our knowledge the first report of this association.
Sh Sadr Eshkevari; A Maboudi; E Akbari Dastak; J Golchai; J Shams Guilani; A Dorjani; N Alizadeh; O Zargari
Volume 8, Issue 2 , 2005, , Pages 104-109
Abstract
Background: Pemphigus is a serious blistering disease with considerable mortality, which heavily burdens on health care system because of the long time hospitalization. It is rare in north America but is considered as the most common immunobullous disease in eastern countries such as Malaysia and China. ...
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Background: Pemphigus is a serious blistering disease with considerable mortality, which heavily burdens on health care system because of the long time hospitalization. It is rare in north America but is considered as the most common immunobullous disease in eastern countries such as Malaysia and China. Objective: The purpose of this study was to define the epidemiologic features of pemphigus in north of Iran. Patients and Methods: One-hundred and twenty-six cases of pemphigus diagnosed based on clinicohistological findings that were hospitalized in Rasht, in north of Iran, between 1995-2001 were studied. Epidemiological data collected from their hospital records were entered in SPSS software and statistical analyses were performed by T-test, Chi-square and Pearson correlation tests. Results: Pemphigus vulgaris was the most common type (83%), followed by foliaceous (6%) and vegetans (1%). The mean age of patients was 45.6 years, with 47.2 in vulgaris and 37.8 in foliaceous group (P<0.05). There was no sex difference in mean age in vulgaris group, whereas the mean age of foliaceous group was 30.2 for females and 51.8 years for males (P<0.05). Female:male ratio was 1.5:1, with 1.44:1 for vulgaris and 1.85:1 for foliaceous groups, respectively. The oral mucosa was the first site of involvement in 63% of vulgaris cases, and 72.4% of them had widespread mucocutaneous disease at the time of hospitalization. Frequency of illness in spring (32%) was higher than other seasons. The mean time for 80% improvement of lesions in hospital was significantly higher in cases with widespread mucocutaneous lesions than cases with only skin or mucosal illness (P<0.05). Conclusion: These findings clearly demonstrate the need for continuous training of involved health workers (Dermatologists, dentists, otolaryngologists, general physicians) for early diagnosis and referral of disease, which not only could decrease the morbidity and mortality, complications rates for patients but also hospitalization costs for patients and health care system.
