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1 J Korean Med Ophthalmol Otolaryngol Dermatol 2016;29(2): pissn eissn Case Report / 증례전신소양감을동반한심상성천포창치험 1 례 이성은 윤화정 고우신동의대학교한의과대학안이비인후피부과학교실 A Case of Pemphigus vulgaris with Generalized Pruritus Sung-Eun Lee Hwa-Jung Yoon Woo-Shin Ko Dept. of Oriental Medical Opthalmology & Otolaryngology & Dermatology, Don-eui university Abstract Objectives : The purpose of this report is to know the effect of Korean medical treatments on pemphigus vulgaris with generalized pruritus. Methods : We treated a 44-years-old woman patient with pemphigus vulgaris on the whole body with Korean medicine. After Korean medical treatment for 17 weeks, we measured the extinction of blisters and decrease of pruritus. We recorded pictures of changes on symptoms. Results & Conclusions : The symptoms of pemphigus vulgaris were significantly improved. The blisters and itching were decreased and skin damages were almost recovered. Thus Korean medical treatments are effective on pemphigus vulgaris. Key words : Pemphigus vulgaris; Generalized Pruritus; Korean medical treatment c 2016 the Society of Korean Medicine Ophthalmology & Otolaryngology & Dermatology This is an Open Access journal distributed under the terms of the Creative Commons Attribution Non-Commercial License ( which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. 112

2 이성은외 2 인 : 전신소양감을동반한심상성천포창치험 1 례 Ⅰ. 서론 천포창 (pemphigus) 은교소체 cadherin인 desmoglein에대한자가항체가생겨표피의각질형성세포들간의접착이소실되어수포가발생되는자가면역성수포성질환이다 1). 대부분의환자가혈청내에각질세포에존재하는항원에대한자가항체를가지고있으며, 표피세포를붙들어주는 desmosome이파괴되면서표피가분리되는증상이나타난다. 표피내수포가형성되는위치, 임상소견및자가항원에따라서크게심상성천포창 (pemphigus vulgaris) 과낙엽상천포창 (pemphigus foliaceous) 으로나눌수있다 2). 한의학적으로천포창 ( 天疱瘡 ) 은수포가전신에파급되어시일이지나瘡으로변하는질환으로心火脾濕이內蘊하고外感風熱毒邪가皮膚에침습하여발생한다. 병변은정상피부혹은홍반위에수포가떼지어출현하며, 포피는극히얇고흐물하고파열된것도있다. 포액이초기에는맑았다가점차혼탁해지며혈액을함유하기도하고, 疱를형성할때심한동통이발생할수있다. 疱가궤파되면맑은삼출물이배출되고, 針刺하는것같은통증이동반되며, 미란면을나타내어치료후에도색소침착이발생할수있다 3). Lee의보고 4) 에의하면천포창이한국에서는가장흔한자가면역성수포성질환으로, 인구 10만명당 1 년에 명정도로발생한다고하였다. 현대의학에서는스테로이드제, 면역억제제등이천포창의치료에사용되고있으며, 다양한치험례가학계에보고되고있다. 그러나현재까지천포창과관련된한의학연구와한방적접근은아직까지미비한실정이다. 따라서저자는동의대학교부속한방병원한방안이비인 Corresponding author : Woo-Shin Ko, The Society of Korean Medicine Opthalmology & Otolaryngology & Dermatology, Dong eui University, San 45-1, Yangjeong-2dong, busanjin-gu, Busan, , Korea (Tel : , wsko@deu.ac.kr) Recieved 2016/4/14 Revised 2016/5/10 Accepted 2016/5/17 후피부과에내원하여한방적처치를통해유의한증상호전도를얻은심상성천포창환자치험 1례를보고하고자한다. Ⅱ. 증례 1. 대상 1) 환자 : 천, 44세, 여자 2) 주소증 1 전신에걸쳐심한소양감있음. 2 양측아래팔부위에지름 2cm크기의대수포 3-4 개씩보이며, 다수의소수포보임. 3 양측상, 하지부및腹部와背部에홍반, 소수포, 搔爬로인한가피있음. 3) 발병일 년경, 2003년경출산후전신소양감발생 년 10월경전신소양감심화 4) 과거력 년경고신대병원 ER visit. 항생제알러지로실신하여 inj-tx 년경 L/C H. ER visit. 항생제알러지로실신하여 inj-tx. 5) 가족력없음 6) 望聞問切睡眠 : 전신소양감및수포로인한불편감으로 1시간정도입면食慾 : 不好 ( 밥 2-3숟가락겨우먹음 ) 消化 : 好小便 : 주간 8-9회야간 3-4회快大便 : 1일 1회보통변 100cc 快 113

3 舌診 : 淡紅舌微黃苔脈診 : 沈滑 7) 현병력상기환자는금연, 금주하는마른체구의 44세여자환자로 2000년경첫출산후전신소양감발생하여 L/C 피부과의원에서내복약치료후호전되었으며, 2003년경두번째출산후상기증상재발하여같은치료병행해오던중 2015년 10월경전신소양감심화되어 L/C 피부과의원에서부신피질호르몬제, 항히스타민제복용과스테로이드연고치료를하였다. 증상호전없어 , 본원한방안이비인후피부과에내원하여외래로통원치료하며한약복용하던중, 양측아래팔부위에대수포발생하여더욱적극적인한방처치원해입원하였다. 8) 치료기간 : 본원외래치료 ( 총 2회 ) : 본원입원치료 ( 총 9일 ) : 본원외래치료 ( 총 13 회 ) 2. 치료방법 1) 약물치료환자의증상을경과관찰하며상황에따라처방을변경하였다. 1 외래치료기간 : 十味敗毒散加味 (Table 1) 를 1첩 2 팩 100cc, Tid용법으로복용하였다 : 越婢加朮湯加味 (Table 2) 를 2첩 3 팩 100cc, Tid용법으로복용하였다 龍膽瀉肝湯加味 A(Table 3) 를 2첩 3팩 100cc, Tid용법으로, 白虎湯加味 (Table 4) 를 1첩 2팩, 70cc, Bid용법 (3:30 p.m., 9:30p.m. ) 으로복용하였다 龍膽瀉肝湯加味 B(Table 5) 를 2첩 3팩, 100cc, Tid용법으로복용하였고, 白虎湯加味 (Table 4) 는그대로복용하였다 龍膽瀉肝湯加味 B(Table 5) 그대로복용하였고, 白虎湯加味 (Table 4) 를消風散加味 (Table 6) 으 로변경하여 1첩 2팩, 70cc, Bid 용법으로복용 하였다 龍膽瀉肝湯加味 B(Table 5) 그대로복용하였고, 消風散加味 (Table 6) 를溫淸飮加味 (Table 7) 로 변경하여 1첩 2팩, 70cc, Bid 용법으로복용하 였다 Bid용법으로복용하던약중단하였고, 龍膽瀉肝 湯加味 B(Table 5) 만복용하였다. Table 1. Composition of Sibmipaedok-San Herb Pharmaceutical name 柴胡 Bupleuri Radix 6 金銀花 Lonicerae Flos 6 乾地黃 Rehmanniae Radix 5 獨活 Angelicae Pubescentis Radix 4 白茯苓 Poria cocos 4 防風 Saposhnikoviae Radix 4 川芎 Cnidii Rhizoma 4 桔梗 Platycodi Radix 4 樺皮 Betulae Platyphyllae Cortex 3 茵蔯 Artemisiae Capillaris Herba 3 梔子 Gardeniae Fructus 3 大黃 Rhei Rhizoma 3 甘草 Glycyrrhizae Radix 2 荊芥 Schizonepetae Spica 2 生薑 Zingiberis Rhizoma 2 Total

4 이성은외 2 인 : 전신소양감을동반한심상성천포창치험 1 례 Table 2. Composition of Wolbigachul-tang Herb Pharmaceutical name 石膏 Gypsum 18 金銀花 Lonicerae Flos 10 麻黃 Ephedrae Herba 6 茵蔯 Artemisiae Capillaris Herba 5 白朮 Atractylodis Rhizoma Alba 4 蒼朮 Atractylodis Rhizoma 4 荊芥 Schizonepetae Spica 4 獨活 Angelicae Pubescentis Radix 4 木通 Akebiae Caulis 3 蟬退 Cicadidae Periostracum 3 防風 Saposhnikoviae Radix 3 甘草 Glycyrrhizae Radix 2 Total 66 Table 3. Composition of Yongdamsagan-tangA Herb Pharmaceutical name 金銀花 Lonicerae Flos 10 龍膽草 Gentianae Scabrae Radix 10 柴胡 Bupleuri Radix 8 車前子 Plantaginis Semen 4 梔子 Gardeniae Fructus 4 當歸 Angelicae Gigantis Radix 4 黃芩 Scutellariae Radix 4 赤茯苓 Poria cocos 4 木通 Akebiae Caulis 4 生地黃 Rehmanniae Radix 4 澤瀉 Alismatis Rhizoma 4 燈心 Junci Medulla 2 甘草 Glycyrrhizae Radix 2 瞿麥 Dianthi Herba 2 香附子 Cyperi Rhizoma 2 木香 Helenii Radix 2 連翹 Forsythiae Fructus 2 萹蓄 Polygoni Avicularis Herba 2 Total 74 Table 4. Composition of Baekho-tang Herb Pharmaceutical name 石膏 Gypsum 20 粳米 Oryzae Semen 12 知母 Anemarrhenae Rhizoma 8 麥門冬 Liriopis Tuber 6 地膚子 Kochiae Fructus 5 人蔘 Ginseng Radix 4 甘草 Glycyrrhizae Radix 4 Herb Total 59 Table 5. Composition of Yongdamsagan-tangB Pharmaceutical name 金銀花 Lonicerae Flos 10 龍膽草 Gentianae Scabrae Radix 10 柴胡 Bupleuri Radix 8 車前子 Plantaginis Semen 4 梔子 Gardeniae Fructus 4 當歸 Angelicae Gigantis Radix 4 黃芩 Scutellariae Radix 4 赤茯苓 Poria cocos 4 木通 Akebiae Caulis 4 生地黃 Rehmanniae Radix 4 澤瀉 Alismatis Rhizoma 4 茵蔯 Artemisiae Capillaris Herba 4 燈心 Junci Medulla 2 甘草 Glycyrrhizae Radix 2 瞿麥 Dianthi Herba 2 香附子 Cyperi Rhizoma 2 木香 Helenii Radix 2 連翹 Forsythiae Fructus 2 萹蓄 Polygoni Avicularis Herba 2 Total

5 Table 6. Composition of Sopung-San Herb Pharmaceutical name 石膏 Gypsum 6 生地黃 Rehmanniae Radix 6 當歸 Angelicae Gigantis Radix 6 牛蒡子 Arctii Fructus Semen 4 蒼朮 Atractylodis Rhizoma 4 防風 Saposhnikoviae Radix 4 木通 Akebiae Caulis 4 知母 Anemarrhenae Rhizoma 3 黑胡麻 Sesami Semen Nigrum 3 苦蔘 Sophorae Radix 2 荊芥 Schizonepetae Spica 2 蟬退 Cicadidae Periostracum 2 甘草 Glycyrrhizae Radix 2 Total 48 Table 7. Composition of Oncheong-Eum Herb Pharmaceutical name 金銀花 Lonicerae Flos 10 乾地黃 Rehmanniae Radix 5 白芍藥 Paeoniae Radix Alba 5 川芎 Cnidii Rhizoma 5 當歸 Angelicae Gigantis Radix 5 黃柏 Phellodendri Chinensis Cortex 3 黃連 Coptis japonica Makino 3 梔子 Gardeniae Fructus 3 Total 36 2) 침구치료 일회용스테인리스豪針 ( 동방침구제작소, mm ) 을사용하여, 양측의合谷 (LI4), 足三里 (ST36), 曲 池 (LI11), 外關 (TE5) 의穴位를취하여 20분간留針하였으며, 2회 / 일시술하였다. 침은直刺로자침하였다. 3) 약침치료황련해독탕약침 0.1ml를이용하여양측肝兪 (BL18), 肺兪 (BL13) 에 1회 / 일시술하였다. 4) 양방적치료입원당시시행한 Lab상 RBC ⁶g/ dl, Hemoglobin 11.4g/ dl, Hematocrit 34.0%, U.BLD ++50, U S.G>=1.030, U.WBC 5-10, S.Epi moderate 확인되었다. UA상혈뇨소견보였으나 1 개월전에시행한검사에서도혈뇨있어정밀검사시행했으나 N-S 한기왕력있어, 환자스스로추가진료를거부하였다. 환자스스로복용중이었던약인 prednisolone 2.5mg, Bepotastine besilate 20mg, Olopatadine hydrochloride 10mg, Hydroxyzine HCl 20mg을지속복용하였으며, Mometasone Furoate 을소양감심할때도포하였다. 5) 2차감염예방전신으로수포가점차진행되어궤파될시 2차감염의우려가있어전신 Simple Dressing을 2회 / 일시행하였다. Simple Dressing은 Klenzo 를이용하여환부를소독한뒤, needle로수포를터트리고마른 cotton ball로닦아내었고, 그후에 Betadine Klenzo 로소독한뒤 cotton ball로닦아내어 Infrared를환부에 10분동안照射하였다. 6) 기타치료 Carbon No.3002(Kokento Carbons) 를이용하여환부에 10분동안광선요법을 1회 / 일시행하였다. 7) 피부의관찰일반형광등조도아래에서 iphone6(apple) 800만화소카메라를이용하여촬영하였다. 116

6 이성은외 2 인 : 전신소양감을동반한심상성천포창치험 1 례 3. 치료경과 환자는야간 VAS9-10, 주간 VAS5 강도의전신에걸친소양감을호소하였으며, 외관상후두부발제이하전신에홍반, 수포, 가피가있었다. 양측아래팔부위에는지름 2cm 크기의대수포여러개보였으며양측상, 하지부에총 30개가량의소수포관찰되었다. 소양감으로인해환부를긁으면수포가터져진물이나오기도하였으며, 이로인해잠을거의자지못하였고식사도불량한상태였다 (Fig. 1). 龍膽瀉肝湯加味 A, 白虎湯加味복용후 에는소양감이야간 VAS8-9, 주간 VAS3-4 단계정도완화되었고, 수면시간도 4시간으로연장되었다. 양측아래팔부위의대수포는맑은포액을 2회 / 일궤파시켜야했었던것이 1회 / 일빈도로감소하였고, 소수포가올라오는곳에조이는듯한동통이 VAS4-5 강도로발생한다고하였다. 양측대퇴내측, 겨드랑이후면에총 30개가량의소수포관찰되어궤파시킨후 Simple Dressing 시행하였으며, 농포가있는부위를위주로경피적외선요법및탄소광선요법시행하였다 (Fig. 2-5). 내복약을龍膽瀉肝湯加味 B로바꾸어복용한후 야간소양감이 VAS6으로완화되었으며, 양측아래팔부위의대수포에는장액성내용물이더이상차오르지않았다. 양측손목, 겨드랑이후면, 대퇴내측에총 10-15개의소수포만발생하였고, 다른부위에서는대수포가나타나지않았다. 야간에소양감이발생하면환자는무의식적으로환부를긁게되어다음날아침에는침대시트위에다량의가피, 인설을관찰할수있었으나, 龍膽瀉肝湯加味 B 복용한후에는시트위에떨어지는가피의양이감소하였다 (Fig. 6-8). 주간발생하는소양감에는큰호전이없어白虎湯加味를消風散加味로변경하였고, 龍膽瀉肝湯加味 B은그대로유지하였다 양측아래팔부위의대수포는結痂가생겨새로운피부가차오르고있었 으며, 전신에새로이발생하는소수포의개수는 10개이내로감소하였고, 농포는관찰되지않았다. 내복약변경하여복용하던중환자개인사정으로퇴원하여외래로통원치료하기로하였다. 퇴원후龍膽瀉肝湯加味 B과消風散加味복용하던중, 얼굴에조그만홍반이발생하였고, 소수포 7-8개가량발생하였다 消風散加味를溫淸飮加味로바꾸어복용하였고, 얼굴의홍반은점차색이옅어져암적색이되었으며새로이발생하는소수포의수도감소하였다. 소양감이완화되어溫淸飮加味복용중단하였고, 龍膽瀉肝湯加味 B만복용하였다 (Fig. 9, 10). Ⅲ. 고찰및결론천포창 (pemphigus) 은피부와점막에물집병변이발생하는질환으로서조직학적으로가시세포분리 (acantholysis) 의소견이특징적으로관찰되는자가면역수포성피부질환 (autoimmune bullous dermatosis) 이다 5-7). 각질형성세포사이의결합체 (desmosome) 내의세포유착분자인데스모글레인 (desmoglein) 에대한 IgG 자가면역항체가발생하여, 가시세포분리에의한표피내수포를형성하는만성적인질환으로 8), 치료하지않을경우치명적이다. 천포창의자가항체는각질형성세포의세포막성분중데스모글레인 (desmoglein-1/3) 에결합하여가시세포분리를일으키는데, 그전과정에걸쳐유발기전은아직명확히밝혀지지않았으나세가지가설이존재한다 9,10). 천포창자가항체에의한가시세포분리의기전은자가항체가데스모글레인 (desmoglein) 에결합하여그입체구조를변형시킴으로써데스모글레인의기능이소실되어각질형성세포의분리가일어나가시세포분리 (acantholysis) 가일어난다는가설 11) 과자가항체가데스모글레인에결합함으로써각질형성세포에서 plasminogen activator 가유리된다는가설 12) 그리고 117

7 Fig Fig Fig Fig Fig Fig Fig Fig Fig Fig

8 이성은외 2 인 : 전신소양감을동반한심상성천포창치험 1 례 각질형성세포에서세포내신호전달이일어나 phospholipase C pathway 가활성화되어가시세포분리가일어난다는가설 13) 등이있다. 최근천포창병변에서세포자멸사기전이가시세포분리의발생에관여한다는의견들이제기되고있다 14-16). 노등의연구 17) 에서는세포자멸사는가시세포분리유발에관계가있는것으로생각되며, 그발생기전은외인성경로및내인성경로가모두활성화되어발생한다고보고하였다. 심상성천포창 (pemphigus vulgaris) 의조직학적소견 18) 은기저층상부표피내수포형성과함께수포내에극해리세포가나타나며, 기저세포는인접세포와는분리되어있지만수포의바닥을형성하면서진피와온전하게부착되어있다. 직접면역형광검사소견은상피세포간공간에세포간물질에대한자가항체가존재하며, 주로 IgG이고 IgA, IgM, C3 등은소수에서침착될수있다 19). 현대의학에서심상성천포창의치료로는전신적인부신피질호르몬제의투여와면역억제제로서 Methotrexate, Cyclophosphamide, Azathioprine 등이있으며, 기타 gold therapy, dapsone, 혈장반출법 (plasmapheresis), 부신피질호르몬제의병변내주입등이있으며, 이외에도국소적인대증요법이있다. 부신피질호르몬제의투여로인한합병증이질병자체에의한사망률보다더문제가되고있어, 부신피질호르몬제의단독투여보다는보조제제의병행투여와보조제제의단독투여등이선호되고있다 20,21). 국내연구에서는대부분의환자에서적은용량의스테로이드 ( mg/kg/day) 로도조절이가능하다고하였으며, 면역억제제의조기사용을권장하고있다 22,23). 또한천포창치료에있어서스테로이드제뿐아니라면역억제제의사용으로인한합병증과사망이발생하므로면역억제제사용역시신중해야한다고보고하고있다 24,25). 한의학적으로천포창 ( 天疱瘡 ) 은수포가전신에파급되어시일이지나瘡으로변하는질환으로心火脾 濕이內蘊하고外感風熱毒邪가皮膚에침습하여발생한다고알려져있다. 熱毒內盛, 濕熱交阻, 陰傷胃敗로변증할수있다. 熱毒內盛型은병변이급속히발생하며수포가떼지어나타나는특징이있으며, 凉血淸熱, 利濕解毒하는犀角地黃湯으로치료한다. 濕熱交阻型은홍반수포가산재되어있고, 미란유즙이비교적많은형태이며淸火健脾, 利濕解毒하는除濕胃笭湯을복용한다. 陰傷胃敗型은천포창후기에다수의結痂가형성되었을때로益氣養陰, 和胃解毒하는益胃湯을복용한다 3). 본증례는금연, 금주하는마른체구의 44세여자환자로내원당시전신에걸친극심한소양감을호소하였으며, 후두부발제이하전신에홍반, 수포, 가피가생성되었고, 양측아래팔부위에는지름 2cm크기의대수포여러개보였으며양측상, 하지부에총 30 개가량의소수포관찰되었다. 소양감으로인해환부를긁으면수포가터져진물이나오기도하였으며, 이로인해잠을거의자지못하며식사도불량한상태였다. 환자는 2000년경, 2003년경출산후전신소양감발생하여 L/C 피부과의원에서치료후호전과악화를반복하던중 2015년 10월경전신소양감심화되어 L/C 피부과의원에서부신피질호르몬제, 항히스타민제복용과스테로이드연고치료를하였으나호전없었다. 양측아래팔부위에대수포발생하였고소양감더욱악화되어입원치료시작하였다. 대수포가궤파되면맑은포액이흘러나왔고, 병변부위에발적, 소양감동반되었다. 外科질환의消托補 3 大內治法중毒이盛하고正氣가아직虛하지않은腫瘍에서成膿을촉진하여毒을내보내는透托法을적용시켜야한다고판단하였다. 東醫寶監 內景篇 에서 " 小便淋澁, 莖中作痛, 屬肝經濕熱, 用龍膽瀉肝湯." 라고언급하였듯 이 26) 淸熱利濕하여수포와소양증을개선시킬수있 는龍膽瀉肝湯加味 A를투여하였다. 東醫寶監 外形篇 에서 " 發疹者, 因胃熱助手少陰火, 入于手太陰肺也. 紅點如斑, 生於皮毛之間, 白虎湯, 瀉心湯, 調胃承 119

9 氣湯, 選用之." 라명시된것에기반하여 26) 야간소양감완화를위해淸熱生津, 利濕止癢하는白虎湯加味를추가복용하였다. 입원기간동안祛風解表, 淸熱利濕, 調和營血하는合谷 (LI4), 足三里 (ST36), 曲池 (LI11), 外關 (TE5) 에자침하였고, 淸熱및消炎효능이있는황련해독탕약침을肺兪 (BL13), 肝兪 (BL18) 에각각주입하여면역력증강과함께淸熱利濕을관장하는肝기능을강화시켰다. 또한 2차감염예방을위해 1일 Bid로전신 Simple Dressing 시행하였고, 疱에서膿이나오는것을완화시키기위해 Carbon 광선치료시행하였다. 소양감이완화되어수면시간이연장되었고, 수면의질도개선되었다. 양측아래팔부위의대수포에포액이차오르는빈도가감소하였으나양측대퇴내측, 겨드랑이후면에총 30개가량의소수포가새로이생성되었으며, 대수포및양측손목의소수포 3-4 곳에서농포발생하였다. 이에淸熱利濕하는효능이탁월한茵蔯蒿 4g을加한龍膽瀉肝湯加味 B로탕약변경하여투약하였다. 야간소양감이완화되었으며, 양측아래팔부위의대수포에는장액성내용물이더이상차오르지않았고, 새로운대수포발생하지않았으며, 소수포의발생빈도와양또한감소하였다. 주간소양감에는큰호전이없어 東醫寶監 外形篇 의 " 肺主皮毛, 故因風熱而頭皮燥痒, 生白屑. 消風散主之." 라한것을참고하여 26) 疏風養血, 淸熱除濕하여피부의發疹, 搔癢感을치료하는消風散加味으로변경하였고, 龍膽瀉肝湯加味 B는그대로유지하여투약하였다. 양측아래팔의대수포는結痂가생겨새로운피부가차오르고있었으며, 전신에새로이발생하는소수포의개수는 10개이내로감소하였고, 농포는관찰되지않았다. 퇴원후얼굴에조그만홍반이발생하였고, 소수포 7-8개가량발생하였다. 東醫寶監 內景篇 에제시된黃連解毒湯과四物湯을合한溫淸飮을기본으로하여 26) 淸熱瀉火하는동시에溫補養血시켜주는溫淸飮加味로교체하였고, 龍膽瀉肝湯加味 B은 그대로유지하였다. 얼굴의홍반은점차색이옅어져암적색이되었으며, 새로이발생하는소수포의개수또한감소하여溫淸飮加味중단하였고, 龍膽瀉肝湯加味 B만복용하였다. 이후지속적으로병변부위및소양감감소하였고, 양측상하지의수포가있던병변은落屑되어옅은색소가침착되었다. 炎症性皮疹은紅斑, 丘疹, 水疱, 膿疱, 結痂, 落屑의순서로치유되는경과를나타내며, 본증례에서도이와같은경과확인되어피부병변이치유되었다고판단하였다. 본증례는스테로이드제내복및외용치료를하였으나소양감호전없었고, 후에대수포동반되어양방처치만으로는큰호전이없었던심상성천포창환자에게한방치료로호전을보였다는점에서의의가있다. 그러나환자의치료에진단을위한조직병리학적검사를시행하는것이도움이되지않을것이라판단하였고, 병변을조직검사또는면역형광검사시행없이육안적으로관찰한뒤진단한것에한계가있다. 천포창에대한임상적연구는발생률이낮아환자수가많지않고, 성별, 연령분포, 질병의심각도에따른대조군을찾기힘들며, 대조군을치료하지않고관찰하는것은윤리적으로불가능하기때문에어려움이많다. 또한치료효과나예후를보기위해연구자의조작을통한연구를시행할수없어대부분후향적연구로시행되고있다 2). 천포창에대한한의학적연구는미비하여, 본증례가추후임상에서천포창환자치료에유의한자료가될것으로생각되며, 지속적인임상례보고와한의학적연구가필요할것으로사료된다. References 1. Stanldy JR, Freedberg IM, Eizen AZ, Wolff K, Austen KF, Goldsmith LA, et al. Fitzpatrick s dermatology in general medicine. 120

10 이성은외 2 인 : 전신소양감을동반한심상성천포창치험 1 례 6th ed. New York:McGraw-Hill. 2003: Kim BD, Kim SC. Clinical study of Korean patients with pemphigus. Kor J Dermatol. 2005;43(8): Seo HS, Yoon HJ. Text of Traditional Korean Dermatolog&Surgery. J Korean Med Ophthalmol Otolaryngol Dermatol. 2007: Lee CW. Autoimmune diseases of the skin. 1st ed. Seoul:Academia. 2002: Katz SL. Blistering skin disease. N Engl J Med. 1985;313: McCuin JB, Hanlon T, Mutasim DF. Autoimmune bullous disease: diagnosis and management. Dermatol Nurs. 2006;18: GammonWR, Fine JD, Forbes M, Briggaman RA. Immuno-flourescence on split skin for the detection and differentiation of basement membrane zone autoantibodis. J Am Acad Dermatol. 1992;27: Udey MC, Stanley JR. Pemphigus-disease of antidesmosomal autoimmunity. JAMA. 1999;282: Amagai M. Autoimmunity against desmosomal cadherins in pemphigus. J Dermatol Sei. 1999;20: Amagai M, Klaus-Kovtun V, Stanley JR. Autoantibodis against a novel epithelial cadherin in pemphigus vulgaris, a disease of cell adhesion. Cell. 1991;67: Stanley JR. Update; structure and function of pemphigus vulgaris antigen. J Dermatol. 1997;24: Lo ML, Pannone G, Staibano S, Mignogna MD, Rubini C, Farronato G, et al. Strict correlation between upar and plakoglobin expression in pemphigus vulgaris. J Cutan Pahol. 2002;29: Esaki C, Seishima M, Yamada T, Osada K, Kitajima Y. Pharmacologic evidence for involvement of phospholipase C in pemphigus IgG-induced inositol 1,4,5-triphophate generation, intracellular calcium increase, and plasminogen activator secretion in DJM-2 cells, a squamous cell carcinoma line. J Invest Dermatol. 1995;105: Puviani M, Marconi A, Cozzani E, Pincelli C. Fas ligand in pemhigus sera induces keratinocyte apoptosis through the activation of caspase-8. J invest Dermatol. 2003; 120: Wang X, Bregegere F, Frusic-Zlotkin M, Feinmesser M, Michel B, Milner Y. Possible apoptotic mechanism in epidermal cell acantholysis induced by pemphigus vulgaris autoimmunoglobulins. Apoptosis. 2004;9: Frusic-Zlotkin M, Pergamentz R, Michel B, David M, Minouni D, Bregegere F, et al. The interaction of pemphigus autoimmunoglobulins with epidermal cells: activation of the fas apoptotic pathway and the use of caspase activity for pathogenicity tests of pemphigus patients. Ann NY Acad Sei. 2005;1050: Roh BH, Whang KU, Cho MK, Park UL, Lee JS, Lee CW. A Study of apoptosis in pemphigus vulgaris. Korean J Dermatol. 2007;45(7): Hashimoto K, Shafran KM, Webber PS, Singer KH. Anti-cell surface pemphigus autoantibody stimulates plasminogen activator 121

11 activity of human epidermal cell; A mechanism for the loss of epidermal cohesion and blister formation. J Exp. 1983;157: Lee SS, Oh HS, Lee MH, Kim NI, Haw CR. A case of pemphigus vulgaris associated with eye involvement. The journal of Kyung Hee University medical center. 1987;3(4): Bystryn JC. Adjuvant therapy of pemphigus. Arch Dermatol. 1984;120: Lever WF, Schaunburg LG. Treatment of pemphigu vularis. Arch Dermatol. 1984; 120: Park BS, Chung JH. Treatment of pemphigus. Korean J Dermatol. 1997;35: Seo PG, Choi WW, Chung JH. Pemphigus in Korea: clinical manifestations and treatment protocol. J Dermatol. 2003;30: Huilgol SC, Black MM. Management of the immunobullous disorders Ⅱ, Pemphigus. Clin Exp Dermatol. 1995;20: Carson PJ, Hameed A, Ahmed AR. Influence of treatment on the clinical course of pemphigus vulgaris. J Am Acad Dermatol. 1996;34: Heo J. DongEuiBoGam. Seoul:Dongeuibogamchulpansa. 2010:831,727,464,

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