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Peripheral Ulcerative Keratitis and Necrotizing Scleritis Initiated by Trauma in the Setting of Mixed Cryoglobulinemia
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Title: Peripheral Ulcerative Keratitis and Necrotizing Scleritis Initiated by Trauma in the Setting of Mixed Cryoglobulinemia
Series Title: Case Rep Ophthalmol 2011;2:392–397
Physical Description: Journal Article
Creator: Johnson, Cameron
Ohlstein, Derek
Publisher: Karger, AG
Place of Publication: Basel, Switzerland
Publication Date: 12/12/11
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Abstract: Purpose: To report a case of peripheral ulcerative keratitis and necrotizing scleritis precipitated by trauma in a patient with mixed cryoglobulinemia due to hepatitis C viral infection. Methods: Case report and literature review. Results: A 62-year-old man with a history of mixed cryoglobulinemia developed an episode of necrotizing scleritis and peripheral ulcerative keratitis one month after repair of a traumatic scleral defect with patch grafting. This episode resolved following treatment with high-dose corticosteroids and the patient underwent successful repeat patch grafting along with a free conjunctival autograft. This is the second reported case of necrotizing scleritis and peripheral ulcerative keratitis associated with mixed cryoglobulinemia. Conclusion: Ophthalmologists should be aware of the association between mixed cryoglobulinemia and necrotizing scleritis/peripheral ulcerative keratitis. Patients with this condition experiencing ocular trauma or undergoing ocular surgery should be monitored closely.
Acquisition: Collected for University of Florida's Institutional Repository by the UFIR Self-Submittal tool. Submitted by Cameron Johnson.
Publication Status: Published
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Source Institution: University of Florida Institutional Repository
Holding Location: University of Florida
Rights Management: All rights reserved by the submitter.
System ID: IR00000736:00001

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Case Rep Ophthalmol 2011; 2 :3 92 397 DOI: 10.1159/000334496 Published online: December 12, 2 011 2011 S. Karger AG, Basel ISSN 1663 2699 www.karger.com/cop This is an Open Access article licensed under the terms of the Creative Commons Attribution NonCommercial NoDerivs 3.0 License ( www.karger.com/OA license ), applicable to the online version of the article only. Distribution for non commercial purposes only. Cameron C. Johnson, M D University of Florida, Dep ar t ment of Ophthalmology P.O. Box 100284 Gainesville, FL 32610 (USA) Tel. +1 352 273 7540, E Mail cameron9 @ ufl. edu 392 Peripheral Ulcerative Keratitis and Necrotizing Scleritis I nitiated by Trauma in the S etting of M ixed Cryoglobulinemia Cameron Campbell Johnson a b Derek Hunter Ohlstein a b a University of Florida, Department of Ophthalmology and b North Florida/ South Georgia Veterans Health System Gainesville, Fla. USA Key Words Scleritis P eripheral ulcerative keratitis PUK Mixed cryoglobulinemia Hepatitis C Abstract Purpose: To report a case of peripheral ulcerative keratitis and nec rotizing scleritis precipitated by trauma in a patient with mixed cryoglobulinemia due to hepatitis C viral infection. Methods: Case report and literature review. Results: A 62 year old man with a history of mixed cryoglobulinemia developed an episode of n ecrotizing scleritis and peripheral ulcerative keratitis one month after repair of a traumatic scleral defect with patch grafting. This episode resolved following treatment with high dose corticosteroids and the patient underwent successful repeat patch gr afting along with a free conjunctival autograft. This is the second reported case of necrotizing scleritis and peripheral ulcerative keratitis associated with mixed cryoglobulinemia. Conclusion: Ophthalmologists should be aware of the association between m ixed cryoglobulinemia and necrotizing scleritis/peripheral ulcerative keratitis. Patients with this condition experiencing ocular trauma or undergoing ocular surgery should be monitored closely. Introduction Peripheral ulcer ative keratitis (PUK) is an inflammatory disease which causes destruction of the peripheral corneal stroma and is associated with an epithelial defect. Non infectious cases are often associated with systemic collagen va scular disease and vasculitis. The sy stemic diseases most often associated with autoimmune PUK are

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Case Rep Ophthalmol 2011; 2 :3 92 397 DOI: 10.1159/000334496 Published online: December 12, 2 011 2011 S. Karger AG, Basel ISSN 1663 2699 www.karger.com/cop 393 nodosa, and Churg Strauss syndrome [1] It is not uncommon for these patients to have an associated necrot izing scleritis [2] Below, we report a case of PUK and necrotizing scleritis initiated by trauma in a patient with mixed cryoglobulinemia (MC), a known cause of small blood vessel vasculitis [3] This is only the second case of PUK with associated sclerit is due to cryoglobulinemia that the authors are aware of in the ophthalmic literature, and the first in which an episode was precipitated by trauma. Case Report A 62 year old man presented with a three week history of sharp pain and redness in his left eye episode of PUK in the left eye which was successfully treated with oral steroids one year prior. On physical exam, visual acuity was 20/20 in both eyes. Slit lamp examination revealed a 1.5 mm scleral noted to be inflamed around the area of the foreign body. The cornea exhibited peripheral thinning The patient was taken to surgery for removal of the foreign body, wh ich consisted of a 2 mm 2 mm less than 1 mm piece of porcelain tile. The resultant scleral defect measured 4 mm long and 2.5 mm wide and extended through approximately 90% of the scleral thickness. The surgeon who performed the repair reports that the defect was not a simple laceration, but a crater like defect due to necrosis of the sclera adjacent to the foreign body. It was therefore unamenable to primary closure. Because the structural integrity of the globe appeared to be compromised, a scleral pat ch graft was sutured over the defect and the conjunctiva was closed with vicryl running and interrupted sutures. The patient did well postoperatively and had resolution of his pain by one week after surgery. However, he returned one month after surgery com plaining of a constant left sided headache. Slit lamp examination showed a conjunctival defect with partial melting of the underlying patch graft, creating a desiccated depression through which the choroid was visible. The area around the defect had 4+ inj ection which did not blanch with phenylephrine and was exquisitely tender. The cornea adjacent to the scleral defect had a 1.5 mm 1 mm area of corneal melting ( fig. 1 ). Because infectious scleritis and keratitis could not be excluded the patient was hos pitalized and placed on fortified topical and intravenous antibiotics. Scleral and corneal cultures, a complete blood count, erythrocyte sedimentation rate, anti nuclear antibody titers, anti neutrophil cytoplasmic antibody titers, rheumatoid factor, rapid plasma reagent, and chest X ray were also performed with negative results. After 48 h ours with no improvement, treatment with intravenous methylprednisolone 1 mg/kg was initiated. 24 h ours later the patient reported mild improvement. He was discharged on improved significantly and the PUK had resolved with reepithelialization of the cornea. One month after discharge the eye was quiet enough ( fig. 2 ) to proceed with a repea t scleral patch graft with primary conjunctival closure. Due to the poor quality of the conjunctival tissue around the patch graft, the patient had dehiscence of the conjunctiva which was unsuccessfully treated with resuturing and amniotic membrane transpl ant ation He eventually required a free conjunctival autograft which achieved good results ( fig. 3 ). The oral steroids were slowly tapered without recurrence of the disease. Discussion Cryoglobulins are abnormal antibodies which precipitate from the serum at low temperatures. In the disorder of MC they are composed of monoclonal i mmunoglobulin M with r heumatoid f actor activity against polyclonal i mmunoglobulin G [3]

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Case Rep Ophthalmol 2011; 2 :3 92 397 DOI: 10.1159/000334496 Published online: December 12, 2 011 2011 S. Karger AG, Basel ISSN 1663 2699 www.karger.com/cop 394 Cryoglobulins act as immune complexes and deposit on the endothelium of small and medium s ize blood vessels causing a vasculitis. These proteins have been found to be produced by a monoclonal expansion of B cells in the liver of patients infected with h epatitis C virus (HCV). Currently more than 90% of MC is associated with HCV, while 10 70% o f patients with HCV exhibit MC. The vasculitis caused by MC often affects the skin, peripheral nerves, and kidneys causing palpable purpura, weakness and paresthesia, and membranoproliferative glomerulonephritis, respectively [4] In the eye, posterior pol e manifestations of MC include Purtscher like retinopathy, central serous chorioretinopathy, and central retinal vein occlusion [5] In the anterior segment, corneal deposits of cryoglobulins, requiring treatment with phototherapeutic keratectomy have been reported [6] There have also been reports of scleritis associated with HCV and MC. Ali et al [7] in 1999 described the first case of scleritis associated with MC and HCV in the r heumatologic literature. This patient had previously undiagnosed HCV. Treat ment was initiated with oral corticosteroids, but the patient eventually required treatment with cyclophosphamide to achieve control of his disease. The authors postulated that scleritis may have been precipitated by the fact that the anterior eye is appro ximately 2 C cooler than the rest of the body, causing cryoglobulins to precipitate. Another case of scleritis associated with MC was described in the ophthalmic li terature in 2008 by Rudrarju et al. [8] This patient, who was diagnosed with HCV 4 years pr ior, had isolated non necrotizing anterior scleritis which was controlled with oral prednisone alone The only previously published case of PUK associated with HCV and MC was reported by Kedhar et al [9] in 2007. This patient had 70% corneal thinning asso ciated with a necrotizing scleritis and required cyclophosphamide to achieve disease resolution. were not initially attributed to MC. This is an association of which ophthal mologists controlled with corticosteroids alone. He also differs from previously reported patients in that he had an episode of severe necrotizing scleritis and PUK precipitated by trauma. There have been reports of noninfectious nodular scleritis following trauma [10] and necrotizing sclerokeratitis has been reported following ocular surgery in patients with underlying vasculiti d es [11] However, to our knowledge, this is the fi rst report of a patient with HCV and MC, but no other connective tissue disease, who developed necrotizing sc leritis and PUK after trauma. metabolic disease of heme syn thesis which leads to the accumulation of porphyrins in the body, which in turn cause lesions of sun exposed areas. There have been case reports of scleritis associated with PCT [12, 13] However, this is unlikely to be the and PUK given that it was initiated by trauma and not sun exposure. Also, subsequent to this episode, he developed mild PUK at the 12 with PCT, which has only been reported nasally and temporally in the s un exposed interpalpebral area. Additionally, an episode of PUK prior to the current one did not have an associated scleritis. Isolated PUK has not been reported with PCT. Finally, his PCT had been quiescent, with n o new skin lesions, in the 6 months prior to his presentation, likely due to treatment with serial phlebotomy. The diagnosis of PCT is

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Case Rep Ophthalmol 2011; 2 :3 92 397 DOI: 10.1159/000334496 Published online: December 12, 2 011 2011 S. Karger AG, Basel ISSN 1663 2699 www.karger.com/cop 395 n ot surprising in our patient given that HCV is a risk factor for it, with 69% of patients with PCT in one study being posi tive for HCV [14] Another point to consider is whether the initial surgery was at higher risk for failure due to the inflamed state of the eye when the initial scleral patch graft was placed. There have been several reports of graft necrosis when a homolo gous scleral graft was used for repair of an eye with active inflammation, due to spread of the inflammatory had no uveal prolapse, one could argue that a repair which simply covered the defect with conjunctiva may have resulted in less of an inflammatory response. A patch graft could have been performed later, if necessary, once the eye was quiet. The success of his subsequent graft was likely due to the fact that infl ammation was controlled prior to the surgery [15] This case, along with previously reported cases of PUK and necrotizing scleritis associated with MC, suggests that it is prudent for ophthalmologists to note the presence of MC in HCV positive patients dur ing treatment of ocular trauma and during preoperative evaluation prior to elective surgery. The additional surgical risk, although small, could be discussed with the patient, and the surgeon could monitor the patient more closely postoperatively. This is especially relevant in that it has been shown that early treatment of surgically induced necrotizing sclerokeratitis is essential to preserving vision [11] Disclosure Statement The authors report no conflicts of interest. Sources of Support Funded in part by an unrestricted grant from Research to Prevent Blindness. Publication of this article was funded in part by the University of Florida Open Access Publishing Fund.

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Case Rep Ophthalmol 2011; 2 :3 92 397 DOI: 10.1159/000334496 Published online: December 12, 2 011 2011 S. Karger AG, Basel ISSN 1663 2699 www.karger.com/cop 396 Fig. 1 Necrotizing scleritis and peripheral ulcerative keratitis 1 month after initial surgery to remove foreign body. Fig. 2 Eye after tre atment with high dose corticosteroids.

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Case Rep Ophthalmol 2011; 2 :3 92 397 DOI: 10.1159/000334496 Published online: December 12, 2 011 2011 S. Karger AG, Basel ISSN 1663 2699 www.karger.com/cop 397 Fig. 3 Eye after scleral patch graft with free conjunctival autograft. References 1 Messmer EM, Foster CS: Vasculitic peripheral ulcerati ve keratitis. Surv Ophthalmol 1999;43:379 396. 2 Stern G: Peripheral corneal disease; in Krachmer JH, Mannis MJ, Holland EJ (eds): Cornea, 2 nd e d. Philadelphia, Elsevier 200 5 pp 339 352. 3 Iannuzzella F, Vaglio A, Garini G: Management of hepatitis C virus related m ixed cryoglobulinemia. Am J Med 2010;123:400 408. 4 Charles ED, Dustin LB: Hepatitis C virus induced cryoglobulinemia. Kidney Int 2009;76:818 824. 5 Myers JP, Di Bisceglie AM, Mann ES: Cryoglobulinemia associated with P urtscher like retinopathy. Am J Ophthalmol 2001;131:802 804. 6 Perez VL, Chavala SH, Ahmed M, Chu D, Zafirakis P, Baltatzis S, Ocampo V, Foster CS: Ocular manifestations and concepts of systemic vasculitides. Surv Ophthalmol 2004;49:339 418. 7 Ali Y, Ghafouri M, Weitzman M, McLean R, Bon is P: Refractory scleritis in a patient with cryoglobulinemia and hepatitis C. J Clin Rheumatol 1999;5:371 373. 8 Rudraraju M, Singh V, Cobb SH Douglas D, Dharmendra P, Shen J: Scleritis associated with hepatitis C: a case report and review of literature. Ann Ophthalmol 2008;40:197 199. 9 Kedhar SR, Belair M, Jun AS, Sulkowski M, Thorne JE: Scleritis and peripheral ulcerative keratitis with hepatitis C virus related cryoglobulinemia. Arch Ophthalmol 2007;125:852 853. 10 Smith JR, Chee SP: Nodular anterior scle ritis associated with ocular trauma. Singapore Med J 2001;42: 180.1. 11 precipitating factors and response to treatment. Br J Ophthalmol 1992;76:17 21. 12 Altiparm ak UD, Oflu Y, Kocaoglu FA, Katircioglu YA, Duman S: Ocular complications in 2 cases with porphyria. Cornea 2008;27:1093 1096. 13 Salmon JF, Strauss PC, Todd G, Murray AD: Acute scleritis in porphyria cutanea tarda. Am J Ophthalmol 1990;109:400 406. 14 Jalil S Grady JJ, Lee C, Anderson KE: Associations among behavior related susceptibility factors in porphyria cutanea tarda. Clin Gastroenterol Hepatol 2010;8:297 302 15 Hamill MB: Management of scleral perforation; in Krachmer JH, Mannis MJ, Holland EJ (eds): Co rnea, 2 nd e d. Philadelphia, Elsevier 200 5 pp 1863 1870.