Epidemiologic study of epidermolysis bullosa in Korea / 대한피부과학회지

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.

A case of poorly differentiated malignant lymphoma in the early childhood / 대한피부과학회지

A 19-month old male patient developed mutiple, firm, plum-colored, finger tip to chestnut sized nodules on the left cheek, right preauricular area, both elbow joints, and right wrist. On physical examination, a right cervical lymph node was palpable. Histology from one of the nodules revealed a diffuse infiltration of atypical, hyperchromatic, polymorphous lymphocytes in the dermis and subrutaneous fat. Bone marow aspiration, chest X-ray, ultrasonogram, and computerized tomogram were performed revealing bone marrow and lymph node involvement. The patient was treated with a POG @8617 schedule. Skin lesions markedly improved after one cycle regimen. We present a case of diffuse, ponrly differentiated, malignant lymphocytic lymphoma in the early childhood.

TGF-beta mRNA expression in cultured fibroblasts / 대한피부과학회지

Transforming growth factor beta(TGF-p), initially identified in vlatelet extracts by virtue of its ability to confer anchorage of independent growth and a necplastic phenotype on mesenchymal cells, has subsequently been identified as a potent inh bit or of proliferation in most cells of epithelial origin. The family of TGF-p peptides is currenly onsisted of four subtypes (TGF-pl, p2, p3, p4). Tkiey are initially translated very larged are urssors of approximately 390 amino acids and produced by a wide variety of cell type. iricluding normal cells and tumor cells. TGF-ps promote deposition of extracellular matrix compcineits, facilitate remodeling events during embryonic development, and suppress immune ce 1 fimetion during the inflammatory process. Several nutaneous diseases are characterized typxcessive and progressive fibrosis of the dermis anil subcutaneous tissues. Prominent amon these disorders are progressive systemic sclerosi(PSS) and generalized morphea(GM), is well as the recently described syndrome of diffuse fasciitis eosinophilia(DF), also known; Shulmans syndrome. The hallmark of the pathologic alteration in these disorders is the excessive deposition of collagen and other connenctive iissues. Macromoleculse in the aerriis and/or the subcutaneous and fascial strutures often accompanied by variable degress of hronic inflammatory cell infiltrates. Now we have examined the expression of TGF-b1 mRNA using of northern blot hybridization with specific sequenced cDNA probe in the normal curltured fibroblasts, placental tissues, and fibrosarcorna derived tumor cell line(HT1080). We found that the size of TGF-b mRNA of each specimen was 2.5kb and theres no alteration of its quality.