بررسی ویژگی های دموگرافیک بیماران مبتلا به IgA bullous dermatosis خطی مراجعه کننده به بیمارستان رازی:‌ مراجعه کننده در سال های 85 تا 95

بررسی ویژگی های دموگرافیک بیماران مبتلا به IgA bullous dermatosis خطی مراجعه کننده به بیمارستان رازی:‌ مراجعه کننده در سال های 85 تا 95

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Linear IgA Bullous Dermatosis که به آن بیماری linear IgA نیز گفته می شود، یک بیماری نادر تاولی خودایمنی است که ویژگی آن وجود رسوب خطی IgA در dermoepidermal junction است. این بیماری در کودکان و بزرگسالان رخ می دهد و ویژگی بالینی آن شبیه درماتیت هرپتی فرم است. بروز این بیماری در کشورهای مختلف متفاوت است و کمتر از نیم تا 2.3 بیمار در هر میلیون در سال گزارش شده است. در بزرگسالان معمولا در سنین بالا (بیشتر از 60 سال) بروز می کند. نظر به اینکه این بیماری جزو بیماری های نادر درماتولوژیک محسوب می گردد و مطالعه ای در مورد این بیماری در ایران تاکنون انجام نشده است، بیمارستان رازی به عنوان قطب علمی پوست کشور و مرکز ارجاع بیمارانی از این دست محیطی مناسب برای بررسی ویژگی های بیماران ایرانی مبتلا به این بیماری قلمداد می گردد. لذا در این مطالعه ما بر آن شده ایم که ویژگی های دموگرافیک بیماران ایرانی مبتلا به IgA bullous dermatosis  خطی را مورد بررسی قرار دهیم.
مواد و روش ها:
در این مطالعه که به صورت گذشته نگر در بیمارستان رازی تهران انجام خواهد شد،  پرونده های درمانگاه پمفیگوس بین سالهای 1385 تا 1395 انتخاب خواهد شد. پرونده¬هایی که در قسمت پاتولوژی خود، Direct immunofluorescence مثبت در درمواپیدرمال جانکشن داشته اند، جدا خواهند شد و وارد مطالعه خواهند گردید. پژوهشگر آموزش دیده، داده های موردنیاز مطالعه را از پرونده های استخراج نموده و وارد فرم های از پیش آماده خواهد کرد. داده های در صورت نقصان، با پیگیری تلفنی تکمیل خواهند گردید. سپس داده های وارد نرم افزار SPSS نسخه 18 خواهد شد و مورد آنالیز توصیفی، کمی، کیفی و تحلیلی قرار خواهد گرفت.
نتایج:
در مجموع 28 بیمار بررسی شد. 35.7% بیماران زن و 64.3% بیماران مرد بودند. میانگین سن زمان تشخیص بیماری 19.64± 37.25 سال بود. کمترین سن زمان تشخیص مربوط به کودکی 3 ساله و بیشترین سن مربوط به خانم 80 ساله بود. 14.2% بیماران همزمان دچار بیماری ulcerative colitis نیز بودند. یک بیمار سلیاک و یک بیمار دیگر سرطان معده داشت. در بررسی سابقه دارویی بیماران، یک نفر شروع ضایعات را پس از مصصرف پنی سیلین و یک بیمار دیگر پس از مصرف سولفاسالازین ذکر می کند. نیمی از بیماران با پردنیزولون و داپسون به طور همزمان درمان شدند. 25% تحت درمان تک دارویی با پردنیزولون و 21% تحت درمان تک دارویی با داپسون قرار داشتند.
نتیجه گیری:
با توجه به شیوع اندک بیماری در سرتاسر دنیا، برای شناخت بیشتر این بیماری به مطالعات بیشتری نیاز است. اما با توجه به نتایج این مطالعه به نظر می رسد در مواردی که ضایعات مشکوک هستند، بیمار به درمان روتین پاسخ نمی دهد و یا ضایعات پس از مصرف داروی خاص (مانند ونکومایسین) و یا در همراهی با بیماری های خاص (مانند UC، بدخیمی ها و ...) دچار بروز ضایعات می شود؛ پزشک معالج باید در ذهن خود تشخیص LABD را نیز داشته باشد تا مورد غفلت واقع نشود.
كليد واژه ها :
 LABD- linear IgA Bullous Dermatosis-pemphigus- اطلاعات دموگرافیک- اطلاعات درمانی
مشخصات دانشجو:
نام: حامد چراغعلی
رشته تحصيلي: پزشکی
مقطع: عمومی                   گروه آموزشي: پوست
پست الكترونيك دانشجو: cheraghali.hamed@gmail.com
اساتيد راهنما و داور:
استاد راهنما: دکتر حمیدرضا محمودی
اساتيد مشاور: دکتر بلیغی- دکتر دانش پژوه- دکتر لاجوردی
اساتيد داور: دکتر عزیزپور- دکتر قندی
زمان دفاع :
   روز یکشنبه  تاريخ  17 تیر 1397 ساعت 12 ظهر
مكان دفاع به آدرس:
میدان وحدت- بیمارستان رازی- سالن آمفی تئاتر
اطلاعات به زبان انگليسي
Title:
Evaluation of demographic characteristics of patients with linear IgA bullous dermatosis referring to Razi Hospital: referring from 1385 to 1395
 
Abstract:
Background and aim:
Linear IgA bullous dermatosis or LABD is a rare autoimmune blistering disorder that usually presents with the abrupt onset of tense bullae and inflammatory lesions on the skin. Both children and adults may be affected. Most cases of LABD are idiopathic. However, LABD has also been reported in association with drug exposure. Vancomycin is the pharmacologic agent most frequently associated with LABD.
Children with LABD frequently develop widespread annular or arciform blisters that are often most abundant on the groin, lower abdomen, and medial thighs. In adults, the annular lesions are less commonly detected, and the trunk and extremities are typical sites of involvement. Pruritus is a common symptom of LABD. Also blindness, airway obstruction, and esophageal strictures and other mucosal involvement may have seen in this disease.
The gold standard for the diagnosis of LABD is the detection of linear deposits of IgA with direct immunofluorescence microscopy.
Studies suggest use of dapsone as first-line therapy. The response to therapy is usually evident within days. Sulfapyridine, sulfamethoxypyridazine, and colchicine are additional options for patients who cannot tolerate dapsone therapy.
Immunosuppressive therapy may be employed for patients with LABD that is refractory to dapsone or sulfonamide therapy. Use of a glucocorticoid-sparing agent such as mycophenolate mofetil is recommended when long-term immunosuppression is required.
In Iran, there is no study about this rare dermatologic disease. Razi hospital as a referral center of dermatologic disease in country, has a good potential of data gathering and analysis of LABD in Iran.
 
Methods and Materials:
This retrospective study was conducted on patients with positive direct immunofluorescence microscopy referred to Razi hospital between 2006 to 2016. Medical records of all patients with LABD diagnosis were reviewed and according to prepared questionnaires, we have gathered demographics data such as age, sex, past medical history, drug history and so on. Treatments also have been reviewed.
Data were analyzed with SPSS 18.
 
Results:
Overall, 28 patients were reviewed. Gender distribution was 35.7% female and 64.3% male.  Age average was 37.25±19.64 years. Minimum age of diagnosis was 3 and maximum was 80. About 14.2% of patients reported a history of ulcerative colitis. One patient (3.5%) has celiac disease and also gastric malignancy was seen in one patient. No one reported using of vancomycin before abruption of blisters. One patient used penicillin and the other one had a drug history of sulfasalazine. Half of patients had treated by both dapsone and prednisolone, 25% prednisolone and 21% dapsone as monotherapy.
 
Conclusion:
Due to low prevalence of this disease worldwide, it seems we need more studies on LABD. We suggest in patient with autoimmune blistering disease that doesn’t have a good response to normal treatment, or in patient that has a combination of autoimmune bullous disease and ulcerative colitis, dermatologist considers LABD as a possible diagnosis. Also we need a countrywide database to know the incidence and prevalence of LABD in Iran.
Keywords:
LABD- linear IgA Bullous Dermatosis-pemphigus
 
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