Epidemiologic study of epidermolysis bullosa in Korea / 대한피부과학회지

ABSTRACT

BACKGROUND: Epidermolysis bullosa is a group of inherited disorcier characterized by blistering of the skin as a result of minor trauma. OBJECTIVE: There exist very little epidemiologic data for most of the major and minor subsets of inherited epidermolysis bullosa in Korea. We look forward to obtaining basic data for the pathogenesis and treatment of epidermolysis bullosa. METHOD: We performed 1st and 2nd trial of survey with the help of 28 dermatologic Departments snd 28 pediatric departments of medical colleges and 14 dermatologic dipartments of general hospitals. RESULTS: 68 cases were collected and classified by disease type, sex distribution, age distribution, and regional distribution. In disease type, 42 cases of EB simplex(62%), 8 cases of junctional type(12%), 10 cases of dominant dystrophic type(15%), 8 cases of recessive dytrcphic type(12%) were reported. In most cases males predominnted, but in dominant dystrophic type both sexes were equivalent. EB simplex, junctional type, RDEB were mostly developed in the 1st decade of life, dominant dystrophic type in the 2nd decade of life. In regional distribution, the number of pi tients was outstanding in large cities such as Seoul, Pusan, Taegu, Kwanju. CONCLUSION: Due to many limitations and shortage of techniques, this epidemiologic study may be notgood enough for the total estimated number of epidermolysis bullos. patients, prevalence rate, and therapeutic modalities. Furthe evaluation should be necessary next time.