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1 대한안과학회지 2016 년제 57 권제 5 호 J Korean Ophthalmol Soc 2016;57(5): ISSN (Print) ISSN (Online) Case Report 시신경염과맥락망막염으로나타난눈매독 1 예 Ocular Syphilis Presenting as both Optic Neuritis and Chorioretinitis in both Eyes 김원제 안장환 김명미 Won-Jae Kim, MD, Jang-Hwan hn, MD, Myung-Mi Kim, MD, PhD 영남대학교의과대학안과학교실 Department of Ophthalmology, Yeungnam University College of Medicine, Daegu, Korea Purpose: To report a patient with ocular syphilis, who showed variable ocular manifestations, including optic neuritis and chorioretinitis in both eyes over a short time period. Case summary: 44-year-male visited our clinic for central scotoma in the left eye. The visual acuity was 20/25 in the right eye and 20/40 in the left eye. The fundus of the left eye showed a slightly hyperemic optic disc and multiple yellowish deposits. One week later, visual acuity and fundus lesion improved to 20/20 without a definitive treatment. However, 1 month later, he reported a new deterioration of vision in his right eye to 20/40. The right eye had a relative afferent pupillary defect and the fundus examination showed a blurred optic disc margin. Serological work-up was recommended but the patient refused. He returned 3 weeks later with an improvement in the right eye vision (20/25) and a worsening in the left eye (20/200). The examination revealed an improvement of the previously blurred disc margin in the right eye and newly developed chorioretinitis with vasculitis in the left eye. serological test was performed. The venereal diseases research laboratory titer was 1:32. The fluorescent treponemal antigen absorbance test as positive for IgG and IgM. He was diagnosed with ocular syphilis and referred to the infectious disease department. He was treated with antibiotics. Six months later, the visual acuity was 20/20 in both eyes and the previous fundus lesions had disappeared. Conclusions: Ocular syphilis should be considered in patients with atypical and variable clinical course. J Korean Ophthalmol Soc 2016;57(5): Keywords: Chorioretinitis, Ocular syphilis, Optic neuritis 매독은성매개질환 (sexually transmitted infection, STI) 의하나로서 Treponema pallidum에의해야기되며, 전염성이매우강하다. 1,2 매독환자발생건수는페니실린의보급과매독의위험도에대한인식의증가로 2000년까지지속적으 Received: Revised: ccepted: ddress reprint requests to Myung-Mi Kim, MD, PhD Department of Ophthalmology, Yeungnam University Hospital, #170 Hyeonchung-ro, Nam-gu, Daegu 42415, Korea Tel: , Fax: mmk@med.yu.ac.kr * This study was presented as a poster at the 114th nnual Meeting of the Korean Ophthalmological Society 로감소하여왔으나, 최근인간면역결핍바이러스 (human immunodeficiency virus, HIV) 와관련하여전세계적으로그발생이산발적으로증가추세에있다. 1,3-5 이중눈매독은눈의여러다양한임상소견을동반하여발생할수있어, 대표적인가면증후군 (masquerade syndrome) 의하나로잘알려져있다 따라서비전형적이고다양한임상경과를보이는눈질환환자에서는항상눈매독의가능성을염두에두어야한다. 근래저자들은짧은기간동안한눈에는시신경염을, 다른한눈에는맥락망막염소견을보이며자연호전과악화를동반한비전형적인임상경과의환자에서눈매독을진단하고치료한증례를경험하였기에이를보고하고자한다. c2016 The Korean Ophthalmological Society This is an Open ccess article distributed under the terms of the Creative Commons ttribution Non-Commercial License ( which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. 857

2 - 대한안과학회지 2016 년제 57 권제 5 호 - 증례보고 44세남자환자가 1주일전우연히발견한좌안의중심암점으로안과외래에방문하였다. 이전에특이한전신적또는안과적과거력은없었다. 시력은우안이 20/25, 좌안이 20/40이었으며, 세극등검사에서전안부의특이소견은관찰되지않았다. 안저검사에서좌안의경도의시신경유두충혈과함께망막의노란색점상병변을확인할수있었다 (Fig. 1, ). 시야검사 (Humphrey field analyzer, Carl Zeiss Meditec Inc., Dublin, C, US) 에서좌안의중심암점소견을확인할수있었다 (Fig. 1C). 스펙트럼영역빛간섭단층촬영 (Spectral domain optical coherence tomography, Spectralis, Heidelberg engineering, Heidelberg, Germany) 에서는좌안황반부의광수용체층 (photoreceptor layer) 내분절 (inner segment) 과외분절 (outer segment) 의경계부가불규칙해진모습을관찰할수있었다 (Fig. 1D). 특별한치료없이 1주일뒤좌안의시력 (20/20) 과이전에관찰되었던망막의병변은호전을보였다. 그러나 1달뒤환자는우안의시력저하로다시안과외래를방문하였다. 우안의시력은 20/40이었고, 상대구심성동공운동장애소견이관찰되었다. 세극등검사에서전안부는정상이었다. 안저검사에서우안의시신 경유두부의전반적인충혈과부종소견을확인할수있었다 (Fig. 2). 환자에게혈액검사및치료를시행할것을권유하였으나, 환자는이를거부하고경과관찰을시행하지않았다. 환자는 3주뒤우안의증상은자연호전되었으나, 다시좌안의시력저하가발생하였다고내원하였다. 시력은우안이 20/25, 좌안이 20/200이었고, 이전진료에서관찰되었던우안의상대구심성동공운동장애와시신경유두부의충혈과부종은호전을보였다. 하지만, 좌안의안저에서주변부혈관염을동반한맥락망막염소견이새롭게관찰되었다 (Fig. 3). 환자에게동의를구하여입원후, 전신검사를시행하였다. 혈액검사에서 venereal diseases research laboratory (VDRL) 양성 (titer 1:32), Fluorescent treponemal antigen absorbance test (FT-S) IgG와 IgM에서양성결과를확인하였다. 뇌척수액검사에서는특이소견은보이지않았다. 이에눈매독으로진단하였고, 환자는내과로전원되어 2주간페니실린정맥주사치료를받았다. 치료 6개월뒤, 두눈의시력은 20/20이었고안저소견및환자의주관적인증상도모두호전을보였다. 고찰 매독은대표적인성매개질환으로서, 역사속에서여러 C D Figure 1. Ocular findings at the initial visit. (, ) The fundus photographs at initial visit. () The left eye shows a slightly hyperemic optic disc with multiple yellowish deposits. (C) The visual field test reveals the central scotoma in the left eye. (D) Spectral-domain optical coherence tomography shows a diffuse disruption of the photoreceptor inner segment-outer segment junction line in the macula of the left eye. 858

3 - 김원제외 : 시신경염과맥락망막염으로나타난눈매독 - Figure 2. The fundus photography of both eyes when the patient reported a new deterioration of vision in the right eye. (, ) The fundus photography of the right eye shows a blurred optic disc margin. C Figure 3. The ocular findings when the patient reported a worsening of vision in the left eye. (, ) The fundus photography of the left eye shows chorioretinitis with vasculitis. (C) The fluorescein angiography shows leakage from lesions and retinal vessels. 문학과미술작품에서도그존재를확인할수있다. 1 미국질병통제관리국 (Centers for Disease Control and Prevention, CDC) 에의하면매독은 1940년대페니실린의보급으로그발생이급격하게줄어들었으나, 근래에는 HIV 감염자와관련하여다시증가추세를보이고있다. 5 이러한발생률의 증가로인해눈매독은포도막염의주요원인으로다시금주목받고있다. 하지만, 눈매독은포도막염뿐만아니라눈의어느부위에서나발생할수있으며, 모든매독의임상단계에서발생할수있으므로비전형적인임상경과를보이는눈질환환자에서그가능성을항상의심해보아야한다. 859

4 - 대한안과학회지 2016 년제 57 권제 5 호 - 이전국내에서는공막염, 포도막염, 유리체염, 맥락망막염, 시신경염등다양한형태로나타난눈매독에대한보고가있었다. 6-8,14 하지만본증례와같이짧은시간간격을두고두눈에각각시신경염과맥락망막염의서로다른임상형태로나타난눈매독의증례는없었다. 또하나흥미로운점은초기에두눈에서나타난임상소견이특별한치료없이도자연호전을보였다는것이다. 매독에의한눈질환의이전보고는대부분일반적인치료에도반응을보이지않는환자에서매독을의심하고진단할수있었던경우였다. 6,8,9,12 하지만본증례에서는처음환자가내원하였을때좌안에보였던소견은 1주일뒤경과관찰에서자연소실을보였고, 1달뒤우안에새롭게나타났던시신경염역시 3주뒤에특별한치료없이호전을보였다. randa and mer 15 는이전에 HIV를진단받았던환자에서자연호전을보인급성매독성후극판모양맥락망막염 (acute syphilitic posterior placoid chorioretinitis) 을보고하였는데, 본증례를통해이전의전신질환이없는경우에서도매독에의한눈질환이자연호전을보일수있음을알수있다. 이는마치 1기나 2기매독에서특별한치료없이도환자의소견은몇주안에자연호전을보이지만, 이후에면역기전을피해남은매독이다양한임상양상으로나타나는것과유사하다. 1,2 이를통해서눈매독도특별한치료없이임상경과의호전이있을수있으며, 그만큼눈매독이다양한임상양상으로나타날수있다는것을알수있었다. 이러한이유로임상소견만으로눈매독의진단은쉽지않을수있다. 눈매독의진단에는주로혈액검사를이용하게되는데, 이러한검사에는매독균감염에반응하여만들어진항체를확인하는방법으로세포로부터나온지질물질을항원으로사용하는비매독법 (non-treponemal test) 과매독균을항원으로사용하는매독법 (treponemal test) 으로분류된다. 비매독법에는 VDRL, rapid plasma reagin이, 매독법에는 FT- S와 microagglutination assay (microhemagglutination assay for Treponema pallidum antibodies [MH-TP], Treponema pallidum hemagglutination [TPH]) 가있다. 5 비매독법의경우, 항체가의변화를관찰함으로써치료효과를확인할수있으므로눈매독이의심되는환자에서혈액검사를시행할때는반드시정성검사와정량검사를모두시행하여야한다. 하지만비매독검사는임신, 고령이나다른면역학적질환과관련하여위양성소견이나타날수있으므로, 비매독법에서양성을보인다면매독법검사를통해확진하는것이중요하다. Park et al 12 은망막혈관염과후부판모양맥락망막염으로나타난눈매독에서처음시행한혈액검사에서는음성을보였으나다시시행한검사에서양성을보인증례를보고하였는데, 이를통해검사자는단한번의혈액검사에 서음성소견을보였다고해서매독가능성을완전무시할수없음을기억해야할것이다. 미국 CDC에서는눈매독에있어다음의항목을조언해주고있다. 5 첫째, 매독의위험이높은환자에서항상눈매독의가능성을염두에두어야한다. 둘째, 매독환자의이전 HIV 검사결과가음성또는결과를모르는경우, 반드시다시 HIV 검사를시행한다. 셋째, 안과적증상이없는초기매독환자와혈액검사에서양성을보인매독환자에서는주의깊은신경학적진찰을시행해야한다. 넷째, 안과적증상이있는매독환자에서는빨리안과검진을시행해야한다. 다섯째, 안과적증상이있는매독환자에서는허리천자 (lumbar puncture) 를통한뇌척수액검사를반드시시행해야한다. 여섯째, 눈매독은신경매독에준하여 10-14일동안페니실린항생제치료를시행한다. 이증례환자의경우, 이전의다른전신적인질환이없었고빠른항생제치료를받을수있어서특별한합병증없이시력을회복할수있었지만, 최근에 HIV와관련하여발생한눈매독에서시력예후가좋지않았던보고도많다. 5 따라서다양한임상경과를보이는눈질환환자에서는반드시눈매독을의심하고, 혈액검사를통해서확인을하는것이필요하며한번의혈액검사로매독의가능성을완전히배제할수없음을기억해야한다. 그리고눈매독환자에서는빠른전신항생제치료를시행해야하며, HIV와의관련성도평가해야할것이다. REFERENCES 1) Lafond RE, Lukehart S. iological basis for syphilis. Clin- Microbiol Rev 2006;19: ) Peeling RW, Hook EW 3rd. The pathogenesis of syphilis: the Great Mimicker, revisited. J Pathol 2006;208: ) Shen J, Feng L, Li Y. Ocular syphilis: an alarming infectious eye disease. Int J Clin Exp Med 2015;8: ecollection ) Chen ZQ, Zhang GC, Gong XD, et al. Syphilis in China: results of a national surveillance programme. Lancet 2007;369: ) Chao JR, Khurana RN, Fawzi, et al. Syphilis: Reemergence of an old adversary. Ophthalmology 2006;113: Epub 2006 ug 28. 6) Hyun SH, Lee DY, Nam DH. case of episcleritis as a manifestation of neurosyphilis. J Korean Ophthalmol Soc 2013;54: ) Lee HJ, Kim MJ, Cha DM, et al. case of ocular syphilis presenting as bilateral optic neuritis. J Korean Ophthalmol Soc 2014; 55: ) Kim J, Park KH, Woo SJ. case of syphilitic outer retinitis mimicking acute zonal occult outer retinopathy. Korean J Ophthalmol 2014;28: ) Kim EH, Lee JE case of vitritis as the primary manifestation without retinitis in ocular syphilis with IDS. J Korean Ophthalmol Soc 2006;47: ) Eandi CM, Neri P, delman R, et al. cute syphilitic posterior 860

5 - 김원제외 : 시신경염과맥락망막염으로나타난눈매독 - placoid chorioretinitis: report of a case series and comprehensive review of the literature. Retina 2012;32: ) Lee SY, Cheng V, Rodger D, Rao N. Clinical and laboratory characteristics of ocular syphilis: a new face in the era of HIV coinfection. J Ophthalmic Inflamm Infect 2015;5:26. 12) Park JH, Joe SG, Yoon YH. Delayed diagnosis of ocular syphilis that manifested as retinal vasculitis and acute posterior multifocal placoid epitheliopathy. Indian J Ophthalmol 2013;61: ) Li SY, irnbaum D, Tessler HH, Goldstein D. Posterior syphilitic uveitis: clinical characteristics, co-infection with HIV, response to treatment. Jpn J Ophthalmol 2011;55: ) Cho YW, Kim CR, Han YS, et al. Clinical manifestations of ocular syphilis combined with neurosyphilis. J Korean Ophthalmol Soc 2014;55: ) randa S, mer R. Sequential spontaneous resolution of acute syphilitic posterior placoid chorioretinitis. Eur J Ophthalmol 2015; 25: = 국문초록 = 시신경염과맥락망막염으로나타난눈매독 1 예 목적 : 짧은기간동안한눈에는시신경염을다른한눈에는맥락망막염소견을보이며, 자연호전과악화를보인비전형적인임상경과의환자에서눈매독을진단하고치료한증례를경험하였기에이를보고하고자한다. 증례요약 : 이전의특이과거력이없었던 44 세남자가 1 주일전우연히발견한좌안의중심암점으로내원하였다. 시력은우안이 20/25, 좌안이 20/40 이었다. 안저검사에서좌안의경도의시신경유두충혈과망막의노란색점상병변을확인할수있었다. 특별한치료없이 1 주일뒤환자의시력과안저소견은호전을보였다. 하지만 1 달뒤환자는우안의시력저하로왔는데시력은 20/40 이었다. 우안의상대구심성동공운동장애소견이관찰되며, 안저에서시신경유두부종을확인하였다. 전신검사와치료를권유하였으나, 환자는임의로경과관찰을중단하였다. 환자는 3 주뒤에다시좌안의시력저하를호소하며다시내원하였다. 우안의시력은 20/25 로, 이전의시신경유두부종은호전을보였다. 좌안의시력은 20/200 이었으며안저에서혈관염을동반한맥락망막염소견이관찰되었다. 혈액검사를시행하였고 venereal diseases research laboratory 양성 (titer 1:32), fluorescent treponemal antigen absorbance test IgG 와 IgM 양성결과를확인하였다. 뇌척수액검사에서특이소견은없었다. 환자는눈매독으로진단받고내과로전원되어 2 주간항생제치료를시행받았다. 치료 6 개월뒤두눈의시력은 20/20 이었고, 안저소견및환자의주관적인증상도모두호전을보였다. 결론 : 자연호전과악화를동반한다양한임상경과를보이는눈질환환자에서반드시눈매독을의심하고, 혈액검사를통해서이를확인하는것이필요하다. < 대한안과학회지 2016;57(5): > 861

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