Anchal Jindal; Rushikesh Shah; Neela Patel
Volume 17, Issue 3 , 2014, , Pages 96-100
Abstract
Background: Immunobullous diseases are morphologically heterogeneous and the differentiation between various subtypes is essential for proper treatment and prognosis. The aim of our study was to analyze and correlate clinical, histopathological, and immunofluorescence findings in ...
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Background: Immunobullous diseases are morphologically heterogeneous and the differentiation between various subtypes is essential for proper treatment and prognosis. The aim of our study was to analyze and correlate clinical, histopathological, and immunofluorescence findings in autoimmune bullous diseases.Method: A cross-sectional study was conducted over a period of two years (2010-2012) after receiving the ethics committee approval. Sixty patients who met the inclusion criteria of immunobullous disease were included in the study. Skin biopsy for histopathological and direct immunofluorescence (DIF) examination was taken. DIF was also done in a few of the cases using the salt-split technique. The final diagnosis was based on clinical, histopathological, and DIFfindings.Result: Sixty cases with a clinical diagnosis of autoimmune bullous diseases were evaluated. In 95% of the cases, the histopathological diagnosis was consistent with the clinical diagnosis and in 75% of the cases, the DIF diagnosis was consistent with the clinical diagnosis. A positive relationship was seen between clinical and DIF findings with r = 0.67 and between histopathologic and DIF findings with r = 0.76. DIF positivity was seen in 100% of the cases of bullous pemphigoid (BP), 100% of the cases of pemphigus foliaceus, and 94.7% of the cases of pemphigus vulgaris which was statistically significant with P < 0.05.Conclusion: Our study provides evidence-based guidance for the diagnosis and classification of various immunobullous disorders. DIF test should be done in conjunction with histopathology tomake a definite diagnosis and minimize both false-positive and false-negative results.
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.
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.
