대한내과학회지 : 제 87 권제 6 호 2014 http://dx.doi.org/10.3904/kjm.2014.87.6.765 레이노병을동반한수부의사구종양 (Glomus tumor) 의 1 예 인하대학교의과대학내과학교실 1 류마티스내과, 2 병리학교실, 3 영상의학과학교실 이승호 1 ㆍ박원 1 ㆍ권성렬 1 ㆍ임미진 1 ㆍ최석진 2 ㆍ김여주 3 ㆍ정경희 1 A Case of a Glomus Tumor in the Hand Associated with Raynaud s Disease Seung Ho Lee 1, Won Park 1, Seong Ryul Kwon 1, Mie Jin Lim 1, Suk Jin Choi 2, Yeo Ju Kim 3, and Kyong-Hee Jung 1 Division of 1 Rheumatology, Departments of Internal Medicine, 2 Pathology, and 3 Radiology, Inha University Collage of Medicine, Incheon, Korea Although it is difficult to reach a diagnosis in patients who complain of pain or sensitivity to cold in their hands, Raynaud's phenomenon is most often suspected in such cases. Symptoms of Raynaud s phenomenon include pallor, cyanosis, and redness following cold exposure. Glomus tumors can also increase patients sensitivity to cold. In this case, our patient complained of symptoms indicative of Raynaud s phenomenon. Although treatment of Raynaud s phenomenon improved the symptoms, pain persisted in the fourth finger of the left hand. We diagnosed the patient with a glomus tumor and, after surgical treatment, the patient s symptoms improved. Here, we have also reviewed and discussed a number of reports of glomus tumors associated with Raynaud s disease. Diagnosing a glomus tumor in the hand may take some time, and the diagnosis could be further delayed if accompanied by Raynaud s phenomenon. Although glomus tumors are rare, we suggest that clinical awareness is important for early diagnosis and treatment. (Korean J Med 2014;87:765-770) Keywords: Glomus tumor; Raynaud phenomenon 서론사구종양 (glomus tumor) 은피부, 특히손가락끝의동정맥문합부의특수기관인사구조직체 (glomus apparatus) 에원발하는매우드문양성종양으로대개조갑부 (subungal area) 에발생한다. 국소에서방사하는격렬한동통이특징으로, 특히 야간이나한랭시에통증이증가하고국소적으로핀으로가볍게압박을가하는 Love s pin test에삼차신경통처럼심한통증을호소한다 [1]. 레이노현상 (Raynaud s phenomenon) 은한랭이나심리적변화에의해손가락이나발가락혈관의연축이촉발되고, 허혈발작으로피부색조가창백, 청색증, 발적의변화를보이 Received: 2014. 5. 8 Revised: 2014. 6. 16 Accepted: 2014. 7. 30 Correspondence to Kyong-Hee Jung, M.D., Ph.D. Division of Rheumatology, Department of Internal Medicine, Inha University Hospital, 27 Inhang-ro, Jung-gu, Incheon 400-711, Korea Tel: +82-32-890-3496, Fax: +82-32-890-2237, E-mail: khjung@inha.ac.kr *This work was supported by an INHA UNIVERSRTIY Research Grant. Copyright c 2014 The Korean Association of Internal Medicine This is an Open Access article distributed under the terms of the Creative Commons Attribution - 765 - Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted noncommercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
- The Korean Journal of Medicine: Vol. 87, No. 6, 2014 - 면서통증, 손발저림등의감각변화가동반되는현상이다. 유병률은일반인구의약 10% 정도로알려져있고특별한기저질환이없이나타나는일차성레이노현상 ( 혹은레이노병 ) 과전신경화증, 루푸스, 류마티스관절염등원인질환이동반되는이차성레이노현상으로나뉜다 [2]. 사구종양과레이노병은주로손가락끝의저린감, 통증이있으면서추위에노출되면악화되는유사한임상소견이있다. 레이노병과동반된사구종양에대한보고는매우드물어 4예의국외보고만이있었다. 본저자들은레이노병을진단하고치료하였으나, 국소증상이지속되는환자에서수부사구종양이동반된 1예를경험하였기에이를문헌고찰과함께보고하는바이다. 증례환자 : 52세여자주소 : 왼쪽세번째, 네번째손가락의통증현병력 : 20년전부터찬물에양쪽손을담그거나추위에노출되면저린통증이있었고, 2년전부터는야간에손가락통증이생겼으며추위에노출시악화되었다. 1년전부터는왼쪽세번째, 네번째손가락의통증이악화되어내원하였다. 가족력 : 특이사항없음. 직업력 : 가사도우미과거력 : 특이병력없음. 진찰소견 : 내원당시활력징후는혈압이 106/66 mmhg, 맥박수분당 73회, 호흡수분당 16회, 체온 36.4 였다. 왼쪽세번째, 네번째손가락의통증과압통이있었고네번째손가락손톱끝부분에는자색의색깔변화가관찰되었다 (Fig. 1). 검사실소견 : 말초혈액소견은혈색소 14.5 g/dl, 백혈구 7,050/mm 3, 혈소판 247,000/mm 3, 적혈구침강속도는 20 mm/hr ( 정상범위 0-22 mm/hr) 였다. 혈청생화학검사에서혈액요소질소 17.6 mg/dl, 크레아티닌 0.826 mg/dl, 아스파르테이트아미노전달효소 31 IU/L, 알라닌아미노전달효소 24 IU/L 로정상이었고, 알칼리인산분해효소 433 IU/L ( 정상범위 103-335 IU/L) 로상승되어있었다. 류마티스인자와항핵항체, 항중성구세포질항체는음성이었다. 영상의학소견 : 손의 X선검사는정상소견이었고, 적외선체열감지기 (digital infrared thermal imaging) 검사에서손가락 과발가락의온도가낮았으며 ( 손가락 : 24.23, 발가락 : 25.10 ) 관심영역 (region of interest) 을이용해서측정한손바닥과손가락끝의온도차이는 -5.57 였고발바닥과발가락끝의온도차이는 -5.45 로차이의범위가컸다 (Fig. 2). 레이노스캔에서는검사초반에스파이크가없이커브가서서히증가하면서양손의차이가벌어지는레이노현상에합당한소견이보였다 (Fig. 3) [3]. 손톱주름모세혈관현미경검사 (nailfold capillary microscopy) 는정상이었다. 임상경과및치료 : 임상증상과검사소견으로레이노병을진단하고보온과유발자극의회피및혈액순환제 (mesoglycan sodium) 를사용하며경과를관찰하였다. 손가락전반의통증과저림및적외선체열감지기의손가락과발가락의온도 ( 손가락 : 29.74, 발가락 : 28.16 ), 손바닥과손가락끝, 발바닥과발바닥끝의온도차이는호전을보였으나, 좌측네번째손끝의통증은지속되며 Love s pin test 가양성 소견이었다. 좌측수부자기공명영상에서네번째손가락조갑부에 0.4 cm 0.5 cm 0.4 cm 크기의종물이관찰되었고, T1 영상에서동신호강도를보이고, T2 영상에서고신호강도를보이며, gadolinium으로조영증강한 T1 영상에서더높은고신호강도를나타내는사구종양에합당한소견이었다 (Fig. 4). 수술적제거를하였으며조직검사에서는다수의사구세포들 (glomus cells) 과더불어혈관들이점액기질 (myxoid stroma) 을침범한소견을보였다 (Fig. 5). 수술후왼쪽네번째손가락의통증과압통은호전되었고, 현재레이노병에대해보존적치료를유지중이다. Figure 1. Purple-blue nail discoloration of the medial portion of the left fourth fingertip (black arrow). - 766 -
- Seung Ho Lee, et al. Glomus tumor with Raynaud s disease - A B C D Figure 2. Digital infrared thermal images of temperature differences in both fingers and toes (finger: -5.57, toe: -5.45 ) were compatible with Raynaud s phenomenon (A, C). After treating for Raynaud s phenomenon, the temperature difference in both fingers and toes was diminished (finger: -1.66, toe: -2.88 ) (B, D). Figure 3. A Raynaud scan showed a specific slow progressive pattern, without the initial spike curve in both hands, as well as inhomogeneous radioactive marker uptake in the second to fifth left fingers of a patient with Raynaud s phenomenon. 고 사구종양은 1812년 Wood에의해 통증이있는피하결절 이라고임상적으로처음기술되었고, 1924년 Masson이수 찰 지의조갑하에서얻은종양으로병리학적소견을최초로기술하면서사구종양이라명명하였다. 주로 20-40대연령에서보고되며여성에서호발하고대개단발성이다. 수부의연조직종양의 1% 정도를차지하는매우드문종양으로, 사구체가풍부하게존재하는수지의조갑하, 외벽 (lateral fold), 속질 (pulp) 등에주로생긴다 [1]. 사구종양은심한동통, 압통및냉온에대한민감성 (cold sensitivity) 의삼대증상이주로나타나며, 가끔부종을호소하는경우도있다. 수지의병소는대개 1 cm 이하의작은청자색병변인데, 조갑하에생기는경우에는전형적인증상이항상존재하지는않아정확한부위를찾기가어렵다. 이러한경우조갑의변형이나변색을세밀히관찰하는것이도움되며핀을사용하여병변의위치를확인하는 Love s pin test 가유용할수있다 [1,4]. 사구종양의가장빈번한위치는손톱밑으로, 외관상드러나지않고초기에는뼈의침윤도드물어수부 X선검사에서쉽게관찰되지않는다. 이전의증례들에서도사구종양이수년동안진단이되지않은경우가많았고, 늦은진단으로뼈와조갑부의손상이생기기도하였다. 또한사구종양은크기가작 - 767 -
- 대한내과학회지 : 제 87 권제 6 호통권제 652 호 2014 - A B C Figure 4. A glomus tumor measuring 0.4 cm 0.5 cm 0.4 cm was detected in the nodular lesion (white arrow) at the far distal subungual area of the left fourth fingertip using magnetic resonance imaging. Well-enhanced oval-shaped intermediate signal intensity on a T1-weighted image (A), high signal intensity on a T2-weighted image (B), and strong enhancement after injection of gadolinium on a T1-weighted image (C). A B Figure 5. examination of a hematoxylin and eosin-stained specimen revealed blood vessels lined with normal endothelial cells and surrounded by solid proliferation of round or cuboidal cells with round nuclei and acidophilic cytoplasm (H&E 40, H&E 400) (A, B). 고만져지지않으며발현양상이다양해서신경종, 관절염, 통풍으로오인되기도한다. 그밖에감별해야할진단으로는혈관종, 황색종, 결절종, 봉입체, 낭종, 이물질등이있다 [4,5]. 레이노현상은창백, 청색증, 발적으로변하는특징적인증상과일반혈액검사, 생화학검사, 손톱주름모세혈관현 미경검사, 적외선체열감지기, 레이노스캔등의검사를통해진단할수있다 [2]. 레이노현상은보통대칭적으로발생하고엄지손가락은잘침범하지않으나, 일부손가락특히두번째와세번째가더심한증상을보이기도하여사구종양과같은손가락의종물이이곳에생긴다면감별이어려울 - 768 -
- 이승호외 6 인. 레이노병을동반한수부의사구종양 - Table 1. Summary of reports of glomus tumors associated with Raynaud s disease Case Sex Age (yr) Duration of symptoms Location 1 [our case] F 39 20 yr Right 2nd finger subungual 2 [7] F 43 Many yr Right 4th finger nail bed 3 [8] F 33 13 yr Left 2nd finger ulnar side Size (cm cm) 4 [9] F 36 5 yr Left 4th finger not descriptive 5 [10] F 21 Many yr Right 4th finger not descriptive MRI, magnetic resonance imaging; Sx., symptoms. 0.4 0.5 Hand MRI 0.6 0.6 Hand MRI 0.5 0.5 Diagnosis Treatment Prognosis 수있다 [6]. 본증례를포함하여현재까지국외에보고된레이노현상과동반된사구종양에대한다섯증례들을비교해본결과, 모든증례의환자는 20-40대젊은여성으로증상이있었던기간이수년이상이었다. 자기공명영상이보편화되기이전에발표된증례들에서는임상적으로의심하여수술적치료이후조직학적으로사구종양이진단되었지만, 본증례와 Abdelrahman MH 등의보고에서는수술전사구종양을수부자기공명영상을통해진단후정확한위치를평가하여수술적치료를시행하였다 [7]. 모든증례에서수술적치료이후증상이호전되었고재발은없었다 (Table 1) [7-10]. 손의동통과한랭감을호소하는환자의감별진단은어려운데, 대개레이노현상을먼저염두에두기가쉽다. 드물긴하나레이노현상을의심하는환자에서손가락의국소적통증이지속된다면, 사구종양과같은동반된국소병변을염두에두고자세한병력청취와신체검사그리고영상검사를통하여조기에감별진단하고치료하는것이필요하겠다. 요약본증례는레이노현상으로내원한환자에서네번째손가락끝의국소통증이지속되어수부자기공명영상검사를 이용해사구종양을진단하고수술적치료로호전된경우이다. 레이노병환자에서손의국소적통증이지속된다면, 매우드물긴하나사구종양과같은국소병변의동반에대해서도고려할필요가있다. 중심단어 : 사구종양 ; 레이노현상 REFERENCES 1. McDermott EM, Weiss AP. Glomus tumors. J Hand Surg AM 2006;31:1397-1400. 2. Herrick AL. The pathogenesis, diagnosis and treatment of Raynaud phenomenon. Nat Rev Rheumatol 2012;8:469-479. 3. Kwon SR, Lim MJ, Park SG, Hyun IY, Park W. Diagnosis of Raynaud s phenomenon by (99m) Tc-hydroxymethylene diphosphonate digital blood flow scintigraphy after one-hand chilling. J Rheumatol 2009;36:1663-1670. 4. Hamdi MF. Glomus tumour of fingertip: report of eight cases and literature review. Musculoskelet Surg 2011;95:237-240. 5. Willard KJ, Cappel MA, Kozin SH, Abzuq JM. Benign subungual tumors. J Hand Surg Am 2012;37:1276-1286. 6. Chikura B, Moore TL, Manning JB, Vail A, Herrick AL. Sparing of the thumb in Raynaud s phenomenon. Rheumatology (Oxford) 2008;47:219-221. 7. Abdelrahman MH, Hammoudeh M. Glomus tumor present- - 769 -
- The Korean Journal of Medicine: Vol. 87, No. 6, 2014 - ing as Raynaud s phenomenon. Case Rep Med 2012;2012: 380540. 8. Yelin FS, Fountain EM. Glomus tumor simulating nerve root compression and Raynuad s phenomenon. Case report. J Neurosurg 1968;29:645-647. 9. Stucke K. Raynaud s disease and glomus tumor. Chirurg 1962;33:178-181. 10. Warter J, Moise R. Glomus tumor under the nail and Raynaud s disease. Strasb Med 1953;4:197-206. - 770 -