Epidermolysis Bullosa Registry Data in Iran

BACKGROUND: In many countries, there is no registry system to record data for Epidermolysis Bullosa patients. However, the first steps for establishing a registry system have been taken in Iran. Therefore, we decided to publish it for the first time. METHODS: This was a prospective cross-sectional study. Data was obtained from 538 patients consecutively enrolled in the Iranian Epidermolysis Bullosa Registry, using a detailed instrument created by burn research center of Iran University of Medical Sciences, Tehran, Iran from Jan 2017 to Sep 2017. Patients’ information such as age, gender, address, educational status, parents’ family relationship and pathology result were recorded. Then a physician examined patients focusing on gastrointestinal system, teeth, ophthalmologic disorders, psychological problems and contracture of the upper and lower limbs and any other complaint. Data entered SPSS ver.19 and analyzed using ANOVA and LSD tests. RESULTS: Overall, 538 EB patients were registered in Iran (6.72 patient in 100.000 person) with an approximately equal ratio between males and females. Among 103 patients whose disease type was determined by a pathologist, 78 patients (75.7%) had dystrophic type, 13 (12.6%) junctional, 9 (8.7%) simplex and 3 (2.9%) kindler type. The most common complaint of patients was dysphagia followed by tooth damage. CONCLUSION: We stablished a data registry for EB patients for the first time in Iran. The frequency of EB in Iran is less than many other countries. However, data completion is to be done to include all patients as possible.