Diagnosis: The Eyes Have It

Abstract

A 43-year-old white woman was transferred to the Mayo Clinic for evaluation of bilateral proptosis and decreased vision in the left eye. Eight months before admission, the patient had a sinus infection and was treated with levofloxacin. She developed a marked lower-extremity rash and respiratory failure requiring mechanical ventilation. This was believed to be Stevens-Johnson syndrome, although no biopsy was performed. Six weeks before admission, the patient had sinus surgery for recurrent sinusitis. After the operation, she noted numbness of the upper left gums and left tongue followed by bilateral proptosis, right eye redness, and right upper eyelid ptosis. Three weeks before admission, the patient had fever, hemoptysis, bloody nasal drainage, and a decrease in left eye visual acuity. Treatment with atropine sulfate in the right eye and prednisolone in each eye, presumably for bilateral anterior uveitis, was initiated. The day before admission, the patient was hospitalized locally for evaluation of bilateral proptosis and decreased vision in the left eye. She received intravenous treatments of vancomycin hydrochloride, meropenem, and amphotericin B liposome, with mild improvement. She was then transferred to the Mayo Clinic. The patient's examination was notable for right eye visual acuity of 20/40 and detection of hand motions in the left eye. Intraocular pressure in both eyes and optic nerves were normal. She had bilateral tense proptosis (Figure 1) with mild eyelid erythema. She had numbness over the face in the maxillary division of the left trigeminal nerve. Anisocoria was noted secondary to pharmacologic dilation of the right eye (Figure 1), and an afferent pupillary defect was detected in the left eye. Bilateral anterior uveitis was present. Nares were patent bilaterally. Nasal septum crusting and a septal perforation were present. Cardiac, lung, and abdominal examinations showed normal results. Skin examination showed scattered palpable purpura of the lower extremities (Figure 2).Figure 2Petechial macules and scattered palpable purpura of the lower extremities.View Large Image Figure ViewerDownload Hi-res image Download (PPT) Laboratory values included erythrocyte sedimentation rate (119 mm/h), hemoglobin (9.1 g/dL), white blood cell count (13,000/μL), platelet count (448×103/μL), and creatinine (1.5 mg/dL). Urinalysis showed a red blood cell count of 31 to 40 per high-power field, hyaline casts, and dysmorphic red blood cells exceeding 25%. Chest radiograph showed normal results. Head and orbit magnetic resonance imaging and magnetic resonance angiography showed bilateral proptosis, an enlarged right lacrimal gland, and a left posterior medial orbital wall phlegmon causing both bowing of the left medial rectus muscle and compression of the left optic nerve. Results of further laboratory evaluation included C-reactive protein (238 mg/L; reference range, <3 mg/L), proteinase (>8 U; reference range, <0.4 U), and positivity for cytoplasmic antineutrophil cytoplasmic antibody (1:128). Nasal mucosal biopsy results confirmed the diagnosis of Wegener granulomatosis, and skin biopsy demonstrated necrotizing vasculitis. She was treated with cyclophosphamide and glucocorticoids. Wegener granulomatosis is an idiopathic systemic vasculitis occurring in the United States at a rate of 3 cases per 100,000,1Cotch M.F. Hoffman G.S. Yerg D.E. et al.The epidemiology of Wegener's granulomatosis: estimates of the five-year period prevalence, annual mortality, and geographic disease distribution from population-based data sources.Arthritis Rheum. 1996; 39: 87-92Crossref PubMed Scopus (275) Google Scholar characterized by necrotizing granulomas of the respiratory tract and focal or proliferative glomerulonephritis. Wegener granulomatosis classically presents with upper-airway or pulmonary manifestations and renal involvement. In addition to the kidneys, affected extrapulmonary sites include skin (46%) and eyes (52%).2Hoffman G.S. Kerr G.S. Leavitt R.Y. et al.Wegener granulomatosis: an analysis of 158 patients.Ann Intern Med. 1992; 116: 488-498Crossref PubMed Scopus (2518) Google Scholar The most common ocular findings are proptosis, scleritis, and conjunctivitis.3Haynes B.F. Fishman M.L. Fauci A.S. Wolff S.M. The ocular manifestations of Wegener's granulomatosis: fifteen years experience and review of the literature.Am J Med. 1977; 63: 131-141Abstract Full Text PDF PubMed Scopus (257) Google Scholar We suspect that the episode of rash and respiratory failure occurring 8 months before this admission represented Wegener granulomatosis and not Stevens-Johnson syndrome.