Images in Clinical Medicine
Peripheral Ulcerative Keratitis in Wegener's Granulomatosis
N Engl J Med 2010; 363:e2July 8, 2010
- Article
An otherwise healthy 15-year-old girl reported progressively worsening pain and redness in her left eye during the previous 2 months. The diagnosis of scleritis with peripheral ulcerative keratitis in the left eye (Panel A) was made on the basis of characteristic examination findings of deep violaceous episcleral injection (long arrow), dilated perilimbal vessels with limbal edema (short arrow), and a corneal epithelial defect associated with an area of corneal thinning (arrowhead). Laboratory testing showed a serum C-reactive protein level of 17.1 mg per liter and an erythrocyte sedimentation rate of 115 mm per hour. Testing for antineutrophilic cytoplasmic antibody with cytoplasmic fluorescence was positive. A review of systems uncovered two recent episodes of otitis media that were unresponsive to antibiotics, unintended weight loss, low-grade fevers, fatigue, recurrent epistaxis, nonproductive cough, and shortness of breath, all of which had begun during the previous month. Urine microscopy revealed granular and red-cell casts. Computed tomography of the chest showed numerous nodular parenchymal lesions without cavitation. A diagnosis of Wegener's granulomatosis was made, and treatment was initiated with oral corticosteroids and cyclophosphamide. No ocular-specific treatment was initiated. Over the next several months, the patient's systemic symptoms and sclerokeratitis began to resolve (Panel B). The limbal vessels returned to their normal caliber (Panel C), although corneal guttering persisted (arrow). Her visual acuity remained 20/20.
Christopher T. Hood, M.D.
Careen Y. Lowder, M.D.
Cleveland Clinic, Cleveland, OH
lowderc@ccf.org
