Peripheral Ulcerative Keratitis Associated with HCV-Related Cryoglobulinemia

Abstract

Purpose To describe a case of peripheral ulcerative keratitis associated with type II cryoglobulinemia in the context of Hepatitis C infection. Methods Case report and literature review. Results A 36-year-old male patient presented to our emergency department with unilateral moderate pain in his right eye, associated with redness and photophobia. Medical background elicited a history of Hepatitis C and IV illicit drug abuse. Ocular examination revealed a BCVA of 20/30 of the affected eye and slight discomfort with eye movement. Biomicroscopy revealed a ring shaped peripheral corneal ulcer, with a dense white stromal infiltrate sparing the limbus, plus an adjacent area of mild anterior diffuse nonnecrotizing scleritis. No intraocular inflammation was present. Posterior segment was normal. The patient was placed under oral prednisolone (1 mg/Kg/day) with gradual tapering. A dramatic clinical response occurred, with complete resolution of the clinical condition. Systemic analytic workup aiming at autoimmune and infectious diseases was negative, except for high HCV-viral load and the presence of type 2 cryoglobulins. Conclusion Albeit rare, cryoglobulinemia, even in the absence of systemic vasculitis, should be considered in the differential diagnosis of PUK, when systemic workout for autoimmune diseases is negative.

Ocular Manifestations of Cryoglobulinemia: a Reappraisal

“…There have been no cohort studies aimed at establishing the prevalence and spectrum of ocular manifestations in patients with cryoglobulinemia. Several case reports have described the occurrence of the following ophthalmologic features in association with cryoglobulinemia: keratoconjunctivitis sicca [23,24]; PUK in patients with HCV- [25][26][27][28] or HBV- [29] related cryoglobulinemia; unilateral or bilateral central retinal vein occlusion [30,31]; massive choroidal infarction [32]; scleritis and retinal vasculitis [33]; anterior segment ischemia and neovascularization [34]; central serous chorioretinopathy [35,36]; Purtscher-like retinopathy [37][38][39]; mucosa-associated lymphoid tissue lymphoma of a lacrimal gland [40]; and funduscopic findings of HVS [41,42]. These reports well demonstrate that virtually all ocular tissues can be involved in CV.…”

Peripheral ulcerative keratitis: A review of aetiology and management

“…43 In the case reports reviewed, half of the patients with PUK and HCV infection had associated MC. 44,45,46 Elevated RF levels and reduced levels are C3 and C4 may be present in patients with MC. 43 A rare extrahepatic manifestation of both HCV infection and MC is the development of PUK.…”

“…43 Peripheral ulcerative keratitis develops in chronic HCV infection, with associated necrotising scleritis being a common clinical finding. 44,45,46,47,48,49 The management of choice for HCV infection is interferon-alpha and ribavirin. 43 The patients who were refractory to this have responded well to a combination of interferon-alpha, ribavirin and rituximab.…”

Peripheral ulcerative keratitis as the primary clinical manifestation of type 2 cryoglobulinemia

“…3À6 In addition, in the case of noninfectious causes of PUK, systemic immunosuppression (i.e., 7 days of pulsed intravenous methylprednisolone at a dose of 500À1000 mg/day, or high dose oral prednisolone [60 mg/day], followed by a tapering course based on the clinical response) is recommended. 5…”