病例21　68歲，女性，右眼紅、眼疼、畏光流淚1個月

CASE 21　A 68-year-old female complaining of redness, pain, photophobia and tearing of right eye for 1 month

見圖1-36。See Fig. 1-36.

鑒別診斷

Differential Diagnosis

◎ 蠶食性角膜潰瘍：是一種慢性疼痛性周邊角膜潰瘍性疾病，原因不明。該病早期為角膜緣炎癥和結膜及表層鞏膜的水腫，角膜炎癥早期為灰白色水腫，病變的角膜部位距離角膜緣2~3mm，可在4~12個月快速進展，累及全周角膜。潰瘍被血管化組織覆蓋。潰瘍通常為新月形，最終只殘余混濁的角膜中央島。臨床上根據病情分為兩種類型。第一種類型：單眼發病，癥狀輕到中度，治療效果好。第二種類型：雙眼發病，臨床癥狀重，治療效果差。

◎ Mooren’s ulcer (MU): Mooren’s ulcer is charac t e rized by painful peripheral corneal ulceration of unknown etiology. The disease generally begins with intense limbal inf lammation and swelling in the episclera and conjunctiva. Corneal changes begin 2 to 3 mm from the limbus, f irst appearing as grey swellings that rapidly furrow, and then proceeding circumferentially and centrally over 4 to 12 months. The bed of the furrow becomes vascularized. These ulcers are often described as crescent-shaped and can leave behind either an opaque,edematous central cornea. There are two types: The f irst is usually unilateral, with mild to moderate symptoms, and generally responds well to treatment. The second type is bilateral, with relatively more pain and generally a poor response to therapy.

◎ Terrien角膜邊緣變性：是一種少見的特發性的非炎癥性的角膜周邊變薄，單眼或者不對稱的雙眼發病，可伴隨角膜新生血管、混濁以及脂質沉著。病變可引起角膜逆規性散光。

◎ Terrien marginal degeneration: It is an uncommon but distinct variety of marginal thinning of the cornea. It causes a slowly progressive non-inf lammatory, unilateral or asymmetrically bilateral peripheral corneal thinning and is associated with corneal neovascularization, opacif ication and lipid deposition. Degeneration may lead to a high degree of against-the-rule or oblique astigmatism.

◎ 邊緣性角膜潰瘍：是一種炎癥性疾病，最終引起周邊角膜變薄。該病通常伴隨自身免疫性疾病，包括類風濕性關節炎、結節性多動脈炎、炎癥性腸病、膠原血管病、系統性紅斑狼瘡(SLE)、復發性軟骨炎、多血管炎肉芽腫、Churg-Strauss綜合征。

◎ Peripheral ulcerative keratits (PUK): It is a group of inf lammatory diseases whose f inal common pathway is peripheral corneal thinning. PUK has been associated with many autoimmune disorders, including: Rheumatoid arthritis, polyarteritis nodosa, inf lammatory bowel disease,collagen vascular diseases, systemic lupus erythematosus(SLE), relapsing polychondritis, granulomatosis with polyangiitis, Churg–Strauss syndrome.

病史詢問

Asking History

◎ 疾病的起病及進展。

◎ 是否有類風濕等免疫性疾病。

◎ Asking the history of onset and progression, and all above system diseases like rheumatoid arthritis or other immune system disease.

眼部檢查

Examination

◎ 視力下降。

◎ Visual acuity was decreased.

◎ 裂隙燈檢查：最初表現為周邊角膜灰白色新月形浸潤，繼而上皮缺損，基質融解，發展成為周邊角膜潰瘍，潰瘍起始于角膜緣，向周邊及中央區進展，呈潛掘狀，最后大部分角膜受侵犯，殘余混濁的角膜中央島。

◎ Slit lamp examination: Typically, Mooren’s ulcer begins as a crescent-shaped gray-white inf iltrate in the peripheral cornea, which progresses to epithelial breakdown and stromal melting eventually developing into corneal ulcer.Corneal ulcer that begins near the limbus and progresses circumferentially and centrally. Eventually most of the cornea is invaded, leaving the central island of the cornea cloudy.

實驗室檢查

Lab

◎ 全血檢查：全血細胞計數、紅細胞沉降率、類風濕因子、抗核抗體、抗中性粒細胞胞漿抗體、胸部及骶髂關節X線檢查、肝功能等。

◎ Complete blood test: Blood cell counts, erythr o cyte sedimentation rate, rheumatoid factor, ant i n u clear antibody,antineutrophil cytoplasmic antibodies, X-ray examination of chest and sacroiliac joint, liver enzymes, etc.

◎ 通過微生物學檢查排除感染性角膜炎。

◎ Infectious etiologies should be excluded by appr o p riate microbiology.

診斷

Diagnosis

蠶食性角膜潰瘍。

Mooren’s ulcer.

治療原則

Management

◎ 藥物治療：局部糖皮質激素，局部免疫抑制劑，全身免疫抑制劑。

◎ Medical approaches: Topical corticosteroids, local and systemic immunosuppression.

◎ 手術治療：結膜切除、羊膜移植術、板層或全層角膜移植術。

◎ Surgical approaches: Conjunctival resection, amniotic membrane transplantation, lamellar keratoplasty, and penetrating keratoplasty.

患者教育和預后

Patient Education & Prognosis

◎ 蠶食性角膜潰瘍是一種慢性、痛性、進展潰瘍性角膜炎，此病病因不明確，病程長、易反復。臨床過程以及預后與疾病的類型和程度相關。

◎ Mooren’s ulcer is a painful, progressive, chronic ulcerative keratitis. The course of Mooren’s ulcer is long and repeatedly.The clinical course, response to therapy, and eventual prognosis of this disease are related to the disease pres e ntation.