片眼の結節性強膜炎と網膜下腫瘤性病変をきたしたベーチェット病の1例

A case of Behçet disease with unilateral nodular scleritis and subretinal tumor as initial manifestations

　27歳女性が，5日前からの右眼充血で受診した。陰部潰瘍が5年前にあり，ベーチェット病と診断されていた。2年前に頸部リンパ節結核があった。眼底は正常であったが，右眼に前房蓄膿があった。4週後に結節性強膜炎が右眼に発症し，さらに前房蓄膿が生じた。結節性強膜炎に相当する眼底部位に白色腫瘤があり，網膜剥離が併発していた。真菌または結核性眼内炎を疑い，硝子体手術を行ったが，初診から3か月後に右眼は壊死性強膜炎を経て有痛性眼球癆になり，眼球摘出に至った。病理学的に眼底の白色腫瘤は脈絡膜膿瘍であった。その後左眼にぶどう膜炎が起こり，前房蓄膿、アフタ性口内炎，結節性紅斑が生じ，完全型のベーチェット病と診断が確定した。全経過を通じ，眼病変はコルヒチン，副腎皮質ステロイド，抗結核療法のいずれにも反応しなかった。初発眼症状が非定型的なために診断と治療が困難であったベーチエット病の症例である。

A 27-year-old female presented with hyperemia in her right eye since 5 days before. She had been diag-nosed with Behçet disease due to genital ulcer 5 years before. She had had cervical tuberculous lymphadenitis 2 years before . Her right eye showed hypopyon iritis with normal-looking fundus. Nodular scleritis developed 4 weeks later followed by hypopyon iritis. A white tumor was present in the fundus corresponding to the site of nodular scleritis. Retinal detachment was also present. We suspected fungal or tuberculous endophthalmitis. After vitreous surgery proved tfutile, her right eye was enucleated for painful phthisis bulbi 3 months after her initial visit. The white tumor was identified as choroidal abscess histopathologically. Her left eye later developed uveitis with hypopyon. She was fi-nally diagnosed with complete type of Behçet disease after aphthous ulcer and nodular erythema became manifest. Throughout the course of disease, the ocular lesions failed to respond to systemic colchicine, corticosteroid, or antitu-berculous therapy. This case illustrates that Behçet disease may show atypical ocular lesions as initial manifestations.