강원도 지역 피부질환의 통계적 고찰 ( 1985 - 1989 )

원진형,허원,안성구,이승헌 ( Jin Hyoung Won,Won Hur,Sung Ku Ahn,Seung Hun Lee ) 대한피부과학회 1992 大韓皮膚科學會誌 Vol.30 No.2

Rifampin에 의한 자반증

원진형,허원,안성구,이승헌,이경주 ( Jin Hyoung Won,Won Hur,Sung Ku Ahn,Seung Hun Lee,Kyung Joo Lee ) 대한피부과학회 1991 대한피부과학회지 Vol.29 No.6

Rifampin-induced thrombocytopenia has been recognized as an immunological reaction associated with intermittent high-dose therapy, although rarely seen with daily low-dose regimens. Our patient was a 33-year-old male who was given rifampin 600㎎/day po for the treatment of pulmonary tuberculosis. His platelet count dropped to 16,000/㎟ after 20 doses of rifampin. Purpura were present over the whole body without the presence of other bleeding sites. Rifampin was discontinued. His platelet count returned to normal three days after discontinuation of therapy. With the increasing use of rifampin for the treatment of tuberculosis, clinicians should recognize the possibility of this drug causing such serious immunological reactions as thrombocytopenia, hemolytic anemia, acute renal failure, and shock with daily or intermittent therapy.

피부신경멜라닌증

원진형(Jin Hyoung Won),안성구(Sung Ku Ahn),김수찬(Soo Chan Kim),이승헌(Seung Hun Lee) 대한피부과학회 1992 대한피부과학회지 Vol.30 No.4

Neurocutaneous melanosis is a rare congenital syndrome characterized by the presence of large or multiple congenital pigmented nevi and a benign or maigrant pigment cell tumor of the leptomeninges. The s ndrome is thought to represent an erroi in the morphogenesis of the embryonal neuroectoderm. Even in the absence of melanorra, symptomatic neurocut.aneous melanosis has extremely poor prognosis. We report a case of neiirocutaneous melanosis in 38-year-old male. He was noted at birth to have a giant pigmented nevus on the bathing trunk area. He presneted with severe headache. Brain CT sca n was performed and 4 x 4cm sized well demarcated contrast enhanced mass was seeii in the right frontal lobe. Histopatho.ogically, the tumor was composed of malignant melanoma cells. Multiple skin biopsies werperformed on the giant pigmented nevus and small nevi. All of the specimens revealed patterns typical of cornpound nevi. Dermatologists following patients with large or multiple congenial pigmented nevi should be aware of this condition to aid in prompt diagnosis. (Kor J Dermatol 1992;30(4):558-563)

Maffucci 증후군

원진형(Jin Hyoung Won),허원(Won Hur),최응호(Eung Ho Choi),한승경(Seung Kyung Hann) 대한피부과학회 1993 대한피부과학회지 Vol.31 No.4

Maffucci's syndrome is a congenital, non-hereditary mesodermal d splasia manifested by multiple enchondromas and hemangiomas. Several case reports and reviews indicate that there is a tendency toward malignant transformation of the enchondroma. The incidence of malignancy may be as high as 30%. We report a case of 10-year-old female with multiple hemangiomas on the face, palms, and soles and skeletal chondromatoses of the multiple bones. (Kor J Dermatol 1993;31(4):616-619)

말초 신경아세포종 ( peripheral neuroblastoma )

허원(Won Hur),원진형(Jin Hyoung Won),안성구(Sung Ku Ahn),이원수(Won Soo Lee),이승헌(Seung Hun Lee),최수임(Soo Imm Choi) 대한피부과학회 1993 대한피부과학회지 Vol.31 No.2

A case of peripheral neuroblastoma of a 33-year-old male, which was located on the left buttock, is presented. Histologcally, this tumor demonstrated smaliound cell infiltrations which were arranged in a characteristic rosette pattern and the tumor cells were positively stained with neuron specific enolase. We review the clinical, histopathological ultrastructural and immunohisto chemical characteristics of this rare tumor, as well as the differential diagnosis with other small round cell tumors. (Kor J Dermatol 1993; 31 (2): 253-257)

칼슘과 레티노인산이 각질형성세포의 교소체 형성에 미치는 영향

김수찬(Soo Chan Kim),원진형(Jin Hyoung Won) 대한피부과학회 1994 대한피부과학회지 Vol.32 No.5

교소체(desmosome)는 세포와 세포를 접착시켜주는 접착장치로서 상피, 심근, 뇌막등의 세포막에 존재하며 전자현미경 소견상 세포질내 판(plaque)과 세포막과 세포막 사이의 코어(core)로 구성된 특징적인 형태를 보인다. 세포질내 판은 중간경 필라멘트(intermediate filament)와 연결되어 있으며 이러한 구조는 과도한 장력을 이겨내는데, 적합한 구조로서 교소체는 피부, 자궁, 방광등 물리적 자극을 많이 받는 조직에 특히 많이 있다. SDS-PAGE를 이용하여 고소체를 생화학적으로 분석해보면 판은 4가지 단백으로, 코어는 4가지 당단백으로 구성되어 있으며 중요구성 성분을 보면 판에는 desmoplakin Ⅰ(250KD), desmoplakin Ⅱ(215KD), plakoglobin(85KD) 등이 있고 코어에는 desmoglein Ⅰ(160KD), desmocollin Ⅰ(130KD), desmocollin Ⅱ(115KD)등이 있다. 교소체는 세포와 세포사이를 접착시켜 줄 뿐 아니라 판은 중간경 필라멘트와 연결되어 있음으로써 조직의 정상구조를 유지시켜주는 역할을 하고 있다. 그러므로 교소체에 이상이 생기는 경우 세포와 세포사이의 접착이 풀어지는 현상 즉 극세포해리증(acantholysis)이 일어나게 된다. 피부과에서 극세포 해리증을 유발하는 대표적인 경우로는 두 가지가 있다. 첫째, 자가면역 수포성 질환인 천포창 환자는 혈청내에 교소체단백에 대한 자가항체를 가지고 있으며 이 자가항체가 교소체단백에 결합함으로써 극세포해리증을 유발한다고 알려져 있다. 둘째, 표피의 분화에 장애를 보이고 이각화증(dyskeratosis)를 초래하는 Darier 병이나 편평상피암의 경우에도 극세포해리증을 보인다. 교소체는 한번 생성되어 변화하지 않는 안정된 구조가 아니고 표피세포가 분화되고 이동됨에 따라 판과 코어가 조립되고 해체될 수 있는 다이나믹한 구조로 생각되고 있다. 칼슘(Ca^+^+)이 표피세포의 분화에 중요한 역할을 하는데 표피세포 배양에서 배양액의 칼슘 농도에 의해서 교소체의 생성이 조절된다는 사실이 알려졌다. Hennings와 Holbrook는 전자현미경 관찰을 통해 저농도의 칼슘이 있는 배양액에서는 교소체가 생성되지 않음을 보았다. 또한 유천포창 항원은 저칼슘농도에서 합성이 증가하는데 반해 천포창항원은 고칼슘농도에서만 합성된다고 하였다. 비타민 A와 그 유도체인 레티노이드는 생체와 배양세포 모두에서 각질형성세포의 분화에 큰 영향을 미치며 분화에 장애를 보이는 여러 피부질환에 치료효과를 가지고 있다. Thivolet등에 의하면 비타민 A가 없는 배양액에서는 각질형성세포의 분화가 증가되고 증층을 이루게 되며 천포창 항원은 감소되는데 반하여 비타민A를 배양액에 다시 첨가하면 분화가 감소되고 천포창 항원의 생성이 증가한다고 하였다. 현재까지 칼슘이나 레티노이드의 각질형성세포 분화와 케라틴 단백에 미치는 영향에 대한 연구는 많이 이루어져 왔으나 교소체 형성에 미치는 영향에 대한 연구는 미흡하며 특히 교소체를 구성하는 각 구성 성분에 대한 연구는 없는 형편이다. 본 실험에서는 대표적인 표피분화 조절물질인 칼슘과 레티노이드의 교소체에 대한 조절기능을 보다 자세히 알아보기 위하여 판에 존재하는 desmoplakin(DP)과 코어에 존재하는 desmoglein(DG)에 대한 단클론 항체를 이용하고 또한 새로운 cadherin으로 알려진 교소체의 130KD 단백에 대한 항체를 가진 심상성 천포창(pemphingus vulgaris;PV) 환자의 혈청 및 desmoglein에 대한 항체를 가진 낙엽상 천포창(pemphingus foliaceus;PF) 환자의 혈청과 최근 새롭게 정의된 질환인 암과 동반되어 피부 점막에 수포를 보이며 desmoplakin에 대한 자가 항체를 가진 부종양 천포창(paraneoplastic pemphigus;PNP) 환자의 혈청을 이용하여 형태학적 및 생화학적 연구를 하였다. Background:Desmosomes are adhesive intercellular junctions that form an important component of the junction complexes of epithelial cells. They provide intercellular links between the intermediate filament cytoskeletons of adjacent cells and are thus involved in maintaining the structural integrity of tissues. Objective:Calcium and retinoids are major regulators of epidermal differentiation regulators on desmosomal somponents, i.e., desmoplakin, desmoglein, and pemphigus antigens. Methods:We used monoclonal antibodies against desmoplakin(DP) and desmoglein(DG), and sera from patients with pemphigus vulgaris(PV), pemphigus foliaceus(PF) and paraneoplastic pemphigus(PNP) to study the effects of calcium and retinoic acids, which are major regulators of epidermal differentation, on desmosomal protein formation in human cultured keratinocytes. We performed immunofluorescence, immunoblotting and immunoprecipitation study using human keratinocytes cultured in high calcium media with or without retinoic acid and in low calcium media with or without retinoic acid. Results: 1. In low calcium(0.15mM) media, PV antigen and DG were produced in a small amount and it appeared that these desmosomal proteins was increased and they were assembled at the desmosomal structures located in cell-cell centact margins. 2. PF antigen, which was identical to the DG, were not produced or expressed in cultured keratinocytes even when cultured in high calcium media. 3. PNP antigen and DP were produced in cultured keratinocytes grown in both high and low calcium media but their production was increased in high calcium media and only in high calcium media they were assembled at the desmosomal structures. 4. Retinoic acids induced loosening of cell-cell contacts of cultured keratinocytes and decreased the production of desmosomal proteins. Conclusion:Our results suggests calcium is a major regulator of the production and assembly of desmosomal proteins including pemphigus antigens, but PF sera and monoclonal antibodies against DG show different antigen binding characteristics. It appears that retinoic acids inhibit production of desmosomal proteins. (Kor J Dermatol 1994;32(5):820~831)

Immunoblot법과 배양각질형성세포의 간접면역형광 검사를 이용한 천포창 항원의 검출

김수찬(Soo Chan Kim),원진형(Jin Hyoung Won),안성구(Sung Ku Ahn) 대한피부과학회 1993 대한피부과학회지 Vol.31 No.3

Background: Pemphigus are chronic autoimmune blistering disorcers characterized by acantholysis. In addition to pemphigus vulgaris(PV), the major clinical variarts are pemphigus foliaceus(PF), paraneoplastic pemphigus(PNP) and drug-induced pemphigus(DP). Detection of pemphigus antigen is important for differential diagnosis as well as research work. Most investigators have identified pemphigus antigens by means of immunoprecipitation using metabolically radiolabeled cultured keratinocytes. However, immunorepitation is generally more expensive, hazardous and time-consuming than immunoblotting. Therefore, establishment of the immunoblotting as a standard technique for the detection of the pemphig us antigens is desirable. Objective : To characterized pemphigus antigens by an immunobloting analysis of human epidermal extract and by indirect itnmunofluroscence study using human of cultured keratinocytes as a substraie. Method : We performed imrnunoblotting analysis af sera from patieiits with PV, PF, PNP and DP with human epidermal extract as a source of antigen. Indirect immunof uorescence study was also performed using human keratinocytes cultured in high or low calcium media for detection of pemphigus antigens. Results : In an immunoblotting analysis, all(9/9) PV sera showed secific reactivities with a 130-KD protein and all(5/5) FF sera showed reactivities with a 150-KK protein, which is most likely desmoglein 1. Furthermore, one of nine PV serum also reacted with a 150-KD protein, which seems to be the identical antigen detected in PF. All PNP(3/3) sera showed reactivities with two protein bands, 210KD and 190KD. In our indirect imrnunofluorescence study using culltured human keratinocytes as a substrate, when keratinocytes were grown in low calcium media, no pimphigus antigens could be detected. However, when grown in high calciurn media, pemphigus vulga ris and paraneoplastic pernphigus antigens were present t the cell-cell contact areas with a puncta;e pattern, whereas pemphigus foliaceus antigen was not, presint in keratinocytes even when cultured in high calcium media. Conclusion : Our results suggests (1) immunoblotting analysis is a reliable technique for defining pemphigus antigen and could be a valuable tool for the differentiation of PV, PF and PNP and(2) PF antigen rnay not be expresseden cultured keratinocytes. (Kor J Dermato 1993; 31(3): 380-388)