특발성 경화성외안근염 환자에서 발생한 눈꺼풀의 IgG4 연관경화병증 1예
A Case of IgG4-Related Sclerosing Disease Involving the Eyelid in an Idiopathic Sclerosing Myositis Patient
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목적: 특발성 경화성외안근염 환자에서 눈꺼풀에 발생한 IgG4 연관경화병증 1예를 보고하고자 한다. 증례요약: 51세 여자 환자가 2011년 6월 약 1개월 전부터 시작되어 점차 심화된 좌안 아래눈꺼풀의 발적, 부종 및 압통을 주소로 내원하였다. 환자는 특발성 경화성외안근염으로 면역억제제를 복용중이었다. 내원 당시 안구운동검사상 좌안의 전방향 운동장애가 있었으나안구돌출은 없었고 좌안 아래눈꺼풀 피하에 결절이 촉지되었으며 자기공명영상에서 좌안 외안근의 비대는 있었으나 눈물샘의 이상은관찰되지 않았다. 이후 눈꺼풀 종양 절제 생검을 시행한 결과 대량의 림프형질세포 및 IgG4 양성 림프형질세포의 침윤을 보여 IgG4연관경화병증으로 진단하였으며 이후 면역억제제와 스테로이드를 경구 복용하였고 술후 7개월까지 재발 없이 유지되고 있다. 결론: IgG4 연관경화병증은 눈부속기에 침범하는 경우 눈물샘염 없이 기타 안와 조직만을 침범하는 경우는 매우 드물다. 저자들은 특발성 경화성외안근염 환자에서 눈물샘을 침범하지 않고 눈꺼풀에 국한되어 발생한 IgG4 연관경화병증을 1예 경험하여 보고하는 바이며눈꺼풀 종양의 감별진단시 고려해야 할 것으로 생각한다.
Abstract
Purpose: To report a case of IgG4-related sclerosing disease involving the eyelid in an idiopathic sclerosing myositis patient. Case summary: A 51-year-old woman presented with swelling, redness, and tenderness of the left lower eyelid of 1 month duration had taken an immunosuppressant for idiopathic sclerosing myositis. Eye movements showed limitation all directions but there was no exophthalmos. A palpable mass was noted in the left lower eyelid. The left extraocular muscles were hypertrophied but the lacrimal gland was normal on orbital magnetic resonance imaging. IgG4-related sclerosing disease was confirmed by immunostained biopsy from the left lower eyelid, showing infiltration of IgG4-positive lymphoplasmacytic cells. The patient was given oral steroids and an immunosuppressant and the symptoms did not recur for at least 7months. Conclusions: IgG4-related sclerosing disease involving ocular adnexa usually consists of bilateral lacrimal gland involvement. Additionally, the orbital soft tissue involvement without dacryoadenitis is rare. The authors of the present study report a case of IgG4-related sclerosing disease involving the left lower eyelid in an idiopathic sclerosing myositis patient and should be considered in the differential diagnosis of eyelid masses.
참고문헌 (17)
- Plaza JA, "Orbital inflammation with IgG4-positive plasma cells: manifestation of IgG4 systemic disease", Arch Ophthalmol, 129, pp.421~428 (2011)
- 김고은, "눈부속기를 침범한 Hyper-IgG4 증후군 3예", 대한안과학회지, 51(8), pp.1133~1138 (2010), 대한안과학회
- Mehta M, "Idiopathic fibroinflammatory disease of the face, eye-lids, and periorbital membrane with immunoglobulin G4-positive plasma cells", Arch Pathol Lab Med, 133, pp.1251~1255 (2009)
- Kamisawa T, "A new clinicopathological entity of IgG4-related autoimmune disease", J Gastroenterol, 38, pp.982~984 (2003)
- Kamisawa T, "IgG4-related sclerosing disease", World J Gastroenterol, 14, pp.3948~3955 (2008)
- Cheuk W, "Chronic sclerosing dacryoadenitis: part of the spectrum of IgG4-related sclerosing disease?", Am J Surg Pathol, 31, pp.643~645 (2007)
- Khosroshahi A, "A clinical overview of IgG4-related systemic disease", Curr Opin Rheumatol, 23, pp.57~66 (2011)
- Espinoza GM., "Orbital inflammatory pseudotumors: etiology, differential diagnosis, and management", Curr Rheumatol Rep, 12, pp.443~447 (2010)
- Zen Y, "IgG4-positive plasma cells in inflammatory pseudotumor (plasma cell granuloma) of the lung", Hum Pathol, 36, pp.710~717 (2005)
- Higashiyama T, "A case of extraocular muscle swelling due to IgG4-related sclerosing disease", Jpn J Ophthalmol, 55, pp.315~317 (2011)
- Sato Y, "Ocular adnexal IgG4-related disease has uniform clinicopathology", Pathol Int, 58, pp.465~470 (2008)
- Kamisawa T, "Usefulness of biopsying the major duodenal papilla to diagnose autoimmune pancreatitis: a prospective study using IgG4-immunostaining", World J Gastroenterol, 12, pp.2031~2033 (2006)
- Sato Y, "IgG4-producing marginal zone B-cell lymphoma", Int J Hematol, 88, pp.428~433 (2008)
- Sandanayake NS, "Presentation and Mmanagement of Ppost-treatment Rrelapse in Aautoimmune Ppancreatitis", Iimmunoglobulin G4-Aassociated Ccholangitis, 2009, pp.7, 1089~1096
- Naitoh I, "Autoimmune pancreatitis associated with various extrapancreatic lesions during a long-term clinical course successfully treated with azathioprine and corticosteroid maintenance therapy", Intern Med, 48, pp.2003~2007 (2009)
- Matsuo T, "Immunoglobulin G4 (IgG4)- positive or -negative ocular adnexal benign lymphoid lesions in relation to systemic involvement", J Clin Exp Hematop, 50, pp.129~142 (2010)
- Ishii S, "Whole-body gallium-67 scintigraphic findings in IgG4-related disease", Clin Nucl Med, 36, pp.542~545 (2011)