Journal Title: پاییز 95
Article title: Epidemiology of the Epidermolysis Bullosa Disease in Iran

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Article abstract:
Introduction & Objective: Epidermolysis Bullosa is a group of inherited bullous disorders characterized by massive blistering of the skin developing in response to minor trauma. The presented statistics of this disorder varies a lot in the world and there is not an exact fact about this disease. In a lot of countries, there is not an EB patient registry system and all of these patients are not diagnosed and recorded. Since the first steps of EB patients’ registry have been started in Iran, we decided to collect, analyze and publicize patients’ statistical data in Iran. Materials & Methods: After launching a conclusive EB patient data registry system by Burn Research Center of Iran University of Medical Sciences, we started to call patients to EB patient health house. Patients’ information such as age, gender, address, educational status, parents’ family relation and pathology result were recorded. Then the physician examined the patients’ different parts of the body such as gastrointestinal system, teeth damages, age damages, psychological problems and contracture of upper and lower limbs were registered. This information was analyzed by statistical program. Results: 342 of EB patients have been diagnosed and registered in Iran (4.2 patient in 100.000 person) with an approximately equal ratio between males and females. Most of the patients were in the school age and students and about 11% of them and academic studies. In 82% of them there was a close or far family relation. The majority patients’ diagnosis was based on clinical tests and less than one third had pathological approval, among which 78% of the patients were of dystrophic type, 10% junctional and about 8% of simplex type. Most of the complications included first dysphasia and in the second place tooth damage. Conclusions: Based on this study, the frequency of EB in Iran is less than other countries. Although, the data completion is to be done in order to screen all the patients. The majority of the patients live in Tehran, Isfehan, Khozestan and Fars and the dystrophic type in Iran is significantly more common with a great difference in comparison to other countries. Patients screening and assessment based on pathology leads to an exact disease type determination. It is considered that gradually, registering all the patients, the number of simplex type patients is increased.
Article KeyWords:
Epidemiology, Epidermolysis Bullosa, Iran
Article Authors:

	دکتر سیامک فرخ فرقانی	First Author
	دکتر محمد جواد فاطمی	Other Author	mjfatemi41@gmail.com
	فراز اسد پور	Other Author
	شیرین عراقی	Other Author
	دکتر افشین نوری	Other Author