성인에서 발생한 HLA-A*11:01 및 A*31:01형의 비전형 스티븐스존슨증후군
Adult-onset Atypical Stevens-Johnson Syndrome with HLA-A*11:01 and HLA-A*31:01 Alleles
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목적: 피부 병변 없이 구강 및 안구 점막만 침범한 성인 비전형 스티븐스존슨증후군 1예를 보고하고자 한다. 증례요약: 발열을 동반한 인후염으로 비스테로이드성 소염제, 아세트아미노펜, 세파클러 항생제 등을 포함한 감기약을 5일간 복용한뒤 피부 이상 없이 구강 점막의 까짐과 양안 결막의 심한 충혈 및 가성막이 발생한 33세 남자가 의뢰되었다. 초진 시, 양안 구결막및 검결막의 상피 결손이 뚜렷했다. 비전형 스티븐스존슨증후군을 염두에 두고 국소 안약제를 이용한 보존적 치료를 시작하였고, 복용 약물에 대한 유전적 감수성을 확인하기 위한 HLA 유전자검사상 HLA-A*11:01와 HLA-A*31:01 대립유전자형이 확인되었다. 10개월후 시행한 마이코플라스마 폐렴균의 항체검사상 면역글로불린G 양성, M 음성이었다. 발병 1년 후 자가혈청안약, 경구 또는 국소 스테로이드, 0.1% 사이클로스포린 안약, 항생제 안약 투여 유지하면서 각결막미란은 현저히 호전되었다. 결론: 비전형 스티븐스존슨증후군은 매우 드물지만 구강 및 안구 점막만 침범할 수 있으므로 약물 복용력과 마이코플라즈마 폐렴병발 등의 적절한 병력 청취를 통해 조기에 의심하는 것이 필요하겠다.
Abstract
Purpose: We report a case of adult-onset atypical Stevens-Johnson syndrome (SJS) in which only the oral and ocular mucosae were involved, without skin lesions. Case summary: A 33-year-old man was referred to our institute with recent severe oral mucositis and bilateral conjunctival hyperemia and pseudomembrane formation, which occurred 5 days after taking cold medicine, including a non-steroidal anti-inflammatory drug, acetaminophen and cefaclor, for pharyngitis with fever. At the initial visit, the patient had an epithelial defect of the bulbar and tarsal conjunctiva in both eyes. Conservative treatment using topical eye drops was started, with suspicion of atypical SJS. In addition, HLA-A alleles were typed to assess possible genetic susceptibility to the relevant drugs, and HLA-A*11:01 and HLA-A*31:01 alleles were confirmed. Immunoglobulin (Ig) G against Mycoplasma pneumoniae was positive and IgM was negative at 10 months after the initial onset of disease. One year after the disease onset, autologous serum eye drops, oral and topical steroids, 0.1% cyclosporine eye drops, and antibiotic eye drops were maintained and the corneal and conjunctival erosions were much improved. Conclusions: Although atypical SJS is very rare, it is important to suspect it early by taking a history of any preceding medication or possible concurrent mycoplasma pneumonia, because atypical SJS may affect the oral and ocular mucosae without skin lesions.
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