Necrotizing Anterior Scleritis with kertouveitis in a Patient with Psoriasis: A Case Report

Necrotizing Anterior Scleritis with kertouveitis in a Patient with Psoriasis: A Case Report | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Short Report Necrotizing Anterior Scleritis with kertouveitis in a Patient with Psoriasis: A Case Report Wengail See, Richmond Siazon, Maria Fideliz Paz This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-4370909/v1 This work is licensed under a CC BY 4.0 License Status: Posted Version 1 posted You are reading this latest preprint version Abstract Objective: To present a rare case of necrotizing anterior scleritis with keratouveitis in a patient with psoriasis and to emphasize the importance of early recognition and aggressive systemic therapy to prevent its sequelae. Necrotizing anterior scleritis is a rare and severely debilitating condition in patients with psoriasis. We present the case of a 60-year-old man with a one-week onset of right eye redness, severe eye pain, and tenderness, accompanied by headache, photophobia, and foreign body sensation. The patient had a history of psoriasis for >20 years and was maintained on methotrexate and topical medications, with poor compliance. He was admitted two weeks prior due to COVID-19 infection. Examination of the right eye showed signs of anterior scleritis and kerato-uveitis. Autoimmune and infection screens yielded negative results. He was administered a regimen of topical cyclosporine BID, atropine TID, and oral prednisone at 1 mg/kg/day that was tapered accordingly after clinical response. Follow-up over the course of three weeks revealed decreased eye pain and improved visual acuity from 20/100 to 20/30 in the right eye. This case of necrotizing sclerotic sclerosis occurred in a setting of long-standing psoriasis and psoriatic arthritis with chronic methotrexate use and various environmental triggers. Early recognition and aggressive treatment with systemic and topical immunomodulators are required to prevent the sequelae of this disease. ocular psoriasis necrotizing scleritis keratouveitis psoriatic arthritis case report Figures Figure 1 Figure 2 Figure 3 INTRODUCTION Psoriasis is an immune-mediated chronic inflammatory disease of unknown etiology that affects approximately 1–3% of the world's adult population with various cutaneous and systemic manifestations [ 1 , 2 ]. In contrast, Michalek et al. reported a prevalence of up to 11.4% in some geographical regions [ 3 ]. An association between psoriasis and ocular disorders has long been established. This is postulated to be due to the similarity in skin, conjunctiva, and eyelid histology inherent to their common ectodermal origin and shared genetic predisposition. It is widely accepted that the pathogenesis is immune-mediated, involving the activation of T helper cells and the release of cytokines that cause inflammatory cell infiltration [ 4 ]. Ocular psoriasis commonly manifests as blepharitis, dry eye, conjunctivitis, or uveitis. Ocular manifestations occur in 10 to 12% of patients with psoriasis, whereas its occurrence in psoriatic arthritis (PsA) is higher at 31%; however, Chandran et al. and Cruz et al. have reported higher rates of 65% and 81.4%, respectively [ 1 , 5 ]. Ocular symptoms often develop in men during acute exacerbations of psoriasis, with a reported latency period of more than five years [ 1 , 2 , 4 ]. In certain instances, ophthalmologic manifestations, such as uveitis, precede psoriatic skin lesions and PsA [ 2 ]. Acute unilateral anterior uveitis has been reported to be a well-known, classical inflammatory eye disease of PsA [ 3 ]. However, most of these symptoms are nonspecific or mild, and may be overlooked. In some cases, it can present as severe scleritis that threatens vision and causes visual morbidity, such as vision loss, if it is not recognized and treated early. Posterior and anterior necrotizing scleritis are two severe forms of scleritis that may require systemic immunosuppressants that target tumor necrosis factor-alpha (TNF), a pro-inflammatory cytokine produced by T helper cells, B lymphocytes, and macrophages, in response to inflammation. This is a key element in the immunopathogenesis of psoriasis. High levels of TNF have been found in the skin, joints, and blood plasma of patients with psoriasis, and are directly correlated with disease activity [ 6 ]. Many cases of posterior scleritis associated with psoriasis and psoriatic arthritis (PsA) have been reported; however, data on anterior necrotizing scleritis with inflammation are limited [ 5 ]. Therefore, we present a rare case of necrotizing anterior scleritis with keratouveitis during a flare-up of psoriasis and emphasize the importance of timely intervention to avoid sight-threatening complications. CASE REPORT A 60-year-old Asian man living in the northern Philippines presented with a one week onset of redness in the right eye, severe eye pain (7/10 on the pain scale), tenderness, headache, photophobia, and foreign body sensation. He had a known case of psoriasis for more than 20 years and was maintained on methotrexate and topical medications for psoriasis but with poor compliance. The medical history revealed a previous admission two weeks prior due to COVID-19 infection. Physical examination revealed multiple lichenified scaly patches on the scalp, neck, and elbows, and erythematous lesions with silvery scales, hypopigmented macules, and papules on the trunk (Fig. 1A-B) . A Boutonniere deformity was noted on the left fifth digit with limited flexion and extension of the distal interphalangeal joints, but not tender on palpation (Fig.C, arrow) . Examination of all the nails revealed oil spots, nail pits, and nail deformities (Fig. 1C-D). Ophthalmic examination revealed a VA of 20/100 + 3 in OD, which did not improve on pinhole, and 20/25 + 2 in OS, which improved to 20/20. The IOP was 12mmHg in both eyes. Biomicroscopy of OD showed conjunctival hyperemia, scleral edema, and ischemia with prominent episcleral and deep scleral vessels in the inferonasal area near the limbus ( Figure A ). An adjacent stromal infiltrate with minimal dye uptake was observed (Fig A, arrow) . Anterior chamber evaluation revealed fine keratic precipitates, + 4 cells and flare and an irregular pupil due to posterior synechiae ( Figure B ). The phenylephrine test revealed no blanching of the deep episcleral vessels. A lens grading of NO3-NC3 was noted. The left eye was unremarkable. Funduscopy findings in both eyes were normal. A working diagnosis of sclerokeratouveitis was made. Gram staining, culture, and KOH of corneal scrapings and eye discharge were negative. Hematology revealed anemia and leukocytosis. Urinalysis revealed an increase in WBCs. Inflammatory markers, ESR and CRP were elevated. Rheumatoid factor, ANA, VDRL, and RPR were all negative. Chest radiography and the Mantoux test results were also negative. Oral prednisone was immediately started at 1 mg/kg/day and gradually decreased after a clinical response. The patient was referred to the Rheumatology service, where the use of biological immunosuppressants was contemplated, but was deferred due to financial constraints and a good clinical response to steroids. The application of topical calcipotriol to skin lesions was prescribed by the Dermatology service. Weekly follow-up revealed a gradual decrease in eye pain (3/10), eye redness, and marked improvement in uncorrected visual acuity from 20/100 to 20/30 in the right eye. After three weeks, the anterior chamber was quiet with no synechiae. There was complete resolution of inflammation; however, the area of scleral necrosis appeared thin and translucent, showing a dark choroid underneath it (Fig. 3A-B). The current plan is to control inflammation, prevent recurrences, and avoid complications of excessive scleral thinning that can lead to perforation or scleral rupture. DISCUSSION Necrotizing scleritis is a rare and severe form with the highest proportion of underlying autoimmune conditions (85.7%), an older age of onset (> 45y/o) and the highest rate of complications, in contrast to other types of scleritis [ 7 , 8 ]. Autoimmune diseases, such as rheumatoid arthritis, granulomatosis with polyangiitis (GPA), relapsing polychondritis, SLE, and PsA, are the most commonly associated entities [ 7 , 9 ]. On the other hand, infectious causes are less common and are responsible for 5–10% of cases [ 10 ]. TB infections, which are endemic in the Philippines, were implicated in 4.2% of the cases in a study in an East Asian population [ 11 ]. Other infectious causes include HIV, herpes zoster, syphilis, Pseudomonas aeruginosa, Proteus, and Staphylococcus, with P. aeruginosa being the most common cause of infectious scleritis [ 7 , 8 , 9 ]. It has been suggested that the absence of an autoimmune pathology in necrotizing scleritis should alert the physician of a possible infectious etiology [ 7 ]. Clinically, infectious scleritis closely resembles autoimmune-associated scleritis and may be impossible to distinguish from one another without histopathologic confirmation. Therefore, it is essential to rule out an infectious etiology before initiating corticosteroids, as this can result in catastrophic complications should an infectious process be present. Our case presented features most consistent with necrotizing anterior scleritis and keratouveitis in a patient with active psoriasis and PsA. The demographic data of the patient, a man belonging to an older age group, clearly fit the profile. Although we believe that an autoimmune mechanism is at play, the exact event that triggered the inflammatory cascade was not clearly defined, as he reported no history of trauma or surgery and no foci of infection were identified. Notwithstanding, the generally accepted immunopathogenesis is the overproduction of activated matrix metalloproteinases (MMPs) from inflammatory cells and scleral fibroblasts, which causes the breakdown of proteoglycans and collagen and destroys the scleral wall. T-helper cells and macrophages play important roles in inflammatory processes [ 7 , 11 ]. Furthermore, the highly vascularized nature of episcleral tissue and the choroid beneath the avascular sclera makes it prone to develop vasculitis in autoimmune conditions, where the deposition of immune complexes may lead to more tissue damage. The focal areas of the white sclera observed in our patient represent capillary closure of the episcleral vasculature, which results in infarction and necrosis of the underlying sclera. The combination of severe vasculitis, infarction, necrosis, and choroid exposure causes severe pain and extreme scleral tenderness, consistent with the symptomatology of our patient. Most often adjacent ocular structures, such as the cornea, the ciliary body, and the trabecular meshwork are involved, manifesting as secondary corneal ulceration, peripheral ulcerative keratitis, uveitis, and increased intraocular pressure. These complications were clearly present in our case, except for IOP elevation; however, all were resolved with the initiation of systemic steroids. Although, recurrences have been reported by the Oasis Autoimmune Systemic Inflammatory Infectious Study (OASIS), and most of them were associated with necrotizing scleritis (26.6%) [ 11 ]. Therefore, periodic eye monitoring is imperative as part of the long-term management plan for these patients. Topical NSAIDs are first-line agents but are generally avoided in acute scleritis due to the risk of scleral melt [ 12 ]. In addition, topical corticosteroids can cause delayed wound healing and perpetuate further scleral thinning. For severe non-infectious scleritis, such as in this case, systemic therapy with oral steroids is the preferred first-line treatment [ 7 , 11 ]. The use of cyclosporine as a steroid-sparing agent or adjunctive therapy has been reported to be effective but methotrexate remains the first-choice treatment [ 7 ] However, recent developments have highlighted the successful use of biologics as steroid-sparing drugs to selectively target immune mediators in the inflammatory cascade. Our patient had a recent bout of Covid-19 infection. On presentation, the patient tested negative for RTPCR. However, COVID-19 could have played a role in the exacerbation of psoriasis and its ocular manifestation. Covid-19 disease causes overwhelming inflammation in some patients, also known as cytokine storm, which is more destructive to organs than the disease itself [ 13 ]. In some cases, necrotizing anterior scleritis was reported in two patients who recovered from coronavirus. Both have no underlying autoimmune disorders. Ocular symptoms appeared 2–3 weeks after recovery from COVID-19 and were successfully treated with immunosuppressants and biologic agents [ 14 ]. Although there is no established link that can directly implicate Covid-19 and necrotizing anterior scleritis, it is widely accepted that Covid-19 is a pro-inflammatory condition that could have played a role in the exacerbation of ocular symptoms. SUMMARY Necrotizing anterior scleritis is a rare and severe inflammatory disease, even more rare in the context of psoriasis and PsA. Early recognition and aggressive treatment with systemic oral steroids as first-line agents, together with topical immunomodulators, were effective in controlling inflammation and preventing its sequelae. In addition, a multidisciplinary team of ophthalmologists, rheumatologists, and dermatologists is warranted for the proper long-term management of these cases. Financial disclosures: The authors declare that they have no known competing financial interests or personal relationships that could have influenced the work reported in this study. Consent: The patient consented to the publication and use of all the images in this report. Data Access Statement: All data are contained in the report Declarations Financial disclosures: The authors declare that they have no known competing financial interests or personal relationships that could have influenced the work reported in this study. Consent: The patient consented to the publication and use of all the images in this report. Data Access Statement: All data are contained in the report Conflict of interest: None Author Contributions: WS collected the data and images, WS and RS wrote the manuscript, and MDP critically revised the manuscript. All the authors approved the final version of the manuscript. References Constantin MM, Ciurduc MD, Bucur S, Olteanu R, Ionescu RA, Constantin T, Furtunescu F (2021) Psoriasis beyond the skin: Ophthalmological changes (Review). Exp Ther Med 22(3):981. 10.3892/etm.2021.10413 Epub 2021 Jul 12. PMID: 34345263; PMCID: PMC8311223 Algarni A, Almuqrin A, Alarwan A (2017) Association between psoriasis and ocular disorders: A narrative review. Clin Med Investigations 2(2). https://doi.org/10.15761/CMI.1000126 Chaiyabutr C, Ungprasert P, Silpa-Archa N, Wongpraparut C, Chularojanamontri L (2020) Psoriasis and Risk of Uveitis: A Systematic Review and Meta-Analysis. Biomed Res Int 2020:9308341. 10.1155/2020/9308341 PMID: 32724820; PMCID: PMC7381949 Rajguru JP, Maya D, Kumar D, Suri P, Bhardwaj S, Patel ND (2020) Update on psoriasis: A review. J Family Med Prim Care 9(1):20–24. 10.4103/jfmpc.jfmpc_689_19 PMID: 32110559; PMCID: PMC7014874 Doumazos S, Kandarakis SA, Petrou P, Karagiannis D, Doumazos L, Georgalas I (2022) Posterior Scleritis in a Patient with Psoriasis Masquerading as Acute Angle Closure Glaucoma. Case Rep Ophthalmol 13(3):717–723. 10.1159/000526714 PMID: 36845449; PMCID: PMC9944587 Fraga NA, Oliveira Mde F, Follador I, Rocha Bde O, Rêgo VR (2012 Nov-Dec) Psoriasis and uveitis: a literature review. Bras Dermatol 87(6):877–883. 10.1590/s0365-05962012000600009 PMID: 23197207; PMCID: PMC3699904 Dutta Majumder P, Agrawal R, McCluskey P, Biswas J (2020) Current Approach for the Diagnosis and Management of Noninfective Scleritis. Asia Pac J Ophthalmol (Phila). ;10(2):212–223. 10.1097/APO.0000000000000341 . PMID: 33290287 Dutta Majumder, P., Agarwal, S., Shah, M., Srinivasan, B., K, P., Iyer, G., … McCluskey,P. (2023). Necrotizing Scleritis: A Review. Ocular Immunology and Inflammation , 1–15. https://doi.org/10.1080/09273948.2023.2206898 Gan YK, Ahmad SS, Alexander SM, Samsudin A (2016) Acute anterior necrotizing scleritis: A case report. J Acute Disease 5(5):439–441. https://doi.org/10.1016/j.joad.2016.08.015 Moshirfar M, Ronquillo Y, Infectious S (2023) Jul 24. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan–. PMID: 32809653 Bin Ismail MA, Lim RHF, Fang HM, Wong EPY, Ling HS, Lim WK, Teoh SC, Agrawal R (2017) Ocular Autoimmune Systemic Inflammatory Infectious Study (OASIS)-report 4: analysis and outcome of scleritis in an East Asian population. J Ophthalmic Inflamm Infect 7(1):6. 10.1186/s12348-017-0124-5 Epub 2017 Feb 15. PMID: 28205148; PMCID: PMC5311008 Abdel-Aty A, Gupta A, Del Priore L, Kombo N (2022) Management of noninfectious scleritis. Ther Adv Ophthalmol 14:25158414211070879. 10.1177/25158414211070879 PMID: 35083421; PMCID: PMC8785299 Zanza C, Romenskaya T, Manetti AC, Franceschi F, La Russa R, Bertozzi G, Maiese A, Savioli G, Volonnino G, Longhitano Y (2022) Cytokine Storm in COVID-19: Immunopathogenesis and Therapy. Med (Kaunas) 58(2):144. 10.3390/medicina58020144 PMID: 35208467; PMCID: PMC8876409 Feizi S, Meshksar A, Naderi A, Esfandiari H (2021) Anterior Scleritis Manifesting After Coronavirus Disease 2019: A Report of Two Cases. Cornea 40(9):1204–1206. 10.1097/ICO.0000000000002795 PMID: 34351874; PMCID: PMC8330625 Additional Declarations No competing interests reported. Cite Share Download PDF Status: Posted Version 1 posted You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-4370909","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Short Report","associatedPublications":[],"authors":[{"id":299198678,"identity":"9b23a7ed-07bc-4e94-898e-af076fa9e68d","order_by":0,"name":"Wengail See","email":"","orcid":"","institution":"Ilocos Training \u0026 Regional Medical Center","correspondingAuthor":false,"prefix":"","firstName":"Wengail","middleName":"","lastName":"See","suffix":""},{"id":299198679,"identity":"55b90cb2-263d-45fc-adf5-4f61477197ab","order_by":1,"name":"Richmond Siazon","email":"data:image/png;base64,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","orcid":"","institution":"Ilocos Training \u0026 Regional Medical Center","correspondingAuthor":true,"prefix":"","firstName":"Richmond","middleName":"","lastName":"Siazon","suffix":""},{"id":299198680,"identity":"36b626ca-b4ed-4808-b5c7-837f7e5c0a38","order_by":2,"name":"Maria Fideliz Paz","email":"","orcid":"","institution":"Oftalvist","correspondingAuthor":false,"prefix":"","firstName":"Maria","middleName":"Fideliz","lastName":"Paz","suffix":""}],"badges":[],"createdAt":"2024-05-05 08:33:58","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-4370909/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-4370909/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":56398457,"identity":"a2bee2a1-81ae-42e4-88b6-425efe8dec34","added_by":"auto","created_at":"2024-05-13 15:56:37","extension":"jpeg","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":428876,"visible":true,"origin":"","legend":"\u003cp\u003eSee image above for figure legend\u003c/p\u003e","description":"","filename":"floatimage1.jpeg","url":"https://assets-eu.researchsquare.com/files/rs-4370909/v1/b9097b9be0a2391529379f76.jpeg"},{"id":56398456,"identity":"8f7e9ae7-e926-4368-8ad6-8f4e81aed1d7","added_by":"auto","created_at":"2024-05-13 15:56:37","extension":"jpeg","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":303805,"visible":true,"origin":"","legend":"\u003cp\u003eSee image above for figure legend\u003c/p\u003e","description":"","filename":"floatimage2.jpeg","url":"https://assets-eu.researchsquare.com/files/rs-4370909/v1/892afe03b67ff1a6493f615d.jpeg"},{"id":56398458,"identity":"99f6391e-faae-4c38-b41a-648b736effac","added_by":"auto","created_at":"2024-05-13 15:56:37","extension":"png","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":388118,"visible":true,"origin":"","legend":"\u003cp\u003eSee image above for figure legend\u003c/p\u003e","description":"","filename":"floatimage3.png","url":"https://assets-eu.researchsquare.com/files/rs-4370909/v1/eae2768dfdb40b752b9d8e05.png"},{"id":77580324,"identity":"c796fd6e-b26f-4212-bdf0-ebe463fad212","added_by":"auto","created_at":"2025-03-03 09:39:23","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":1571321,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-4370909/v1/f79f5db4-f26b-4b61-8942-72e30949e2ab.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Necrotizing Anterior Scleritis with kertouveitis in a Patient with Psoriasis: A Case Report","fulltext":[{"header":"INTRODUCTION","content":"\u003cp\u003ePsoriasis is an immune-mediated chronic inflammatory disease of unknown etiology that affects approximately 1\u0026ndash;3% of the world's adult population with various cutaneous and systemic manifestations [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e, \u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e]. In contrast, Michalek et al. reported a prevalence of up to 11.4% in some geographical regions [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e]. An association between psoriasis and ocular disorders has long been established. This is postulated to be due to the similarity in skin, conjunctiva, and eyelid histology inherent to their common ectodermal origin and shared genetic predisposition. It is widely accepted that the pathogenesis is immune-mediated, involving the activation of T helper cells and the release of cytokines that cause inflammatory cell infiltration [\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eOcular psoriasis commonly manifests as blepharitis, dry eye, conjunctivitis, or uveitis. Ocular manifestations occur in 10 to 12% of patients with psoriasis, whereas its occurrence in psoriatic arthritis (PsA) is higher at 31%; however, Chandran et al. and Cruz et al. have reported higher rates of 65% and 81.4%, respectively [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e, \u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e]. Ocular symptoms often develop in men during acute exacerbations of psoriasis, with a reported latency period of more than five years [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e, \u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e, \u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e]. In certain instances, ophthalmologic manifestations, such as uveitis, precede psoriatic skin lesions and PsA [\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e]. Acute unilateral anterior uveitis has been reported to be a well-known, classical inflammatory eye disease of PsA [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e]. However, most of these symptoms are nonspecific or mild, and may be overlooked. In some cases, it can present as severe scleritis that threatens vision and causes visual morbidity, such as vision loss, if it is not recognized and treated early. Posterior and anterior necrotizing scleritis are two severe forms of scleritis that may require systemic immunosuppressants that target tumor necrosis factor-alpha (TNF), a pro-inflammatory cytokine produced by T helper cells, B lymphocytes, and macrophages, in response to inflammation. This is a key element in the immunopathogenesis of psoriasis. High levels of TNF have been found in the skin, joints, and blood plasma of patients with psoriasis, and are directly correlated with disease activity [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eMany cases of posterior scleritis associated with psoriasis and psoriatic arthritis (PsA) have been reported; however, data on anterior necrotizing scleritis with inflammation are limited [\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e]. Therefore, we present a rare case of necrotizing anterior scleritis with keratouveitis during a flare-up of psoriasis and emphasize the importance of timely intervention to avoid sight-threatening complications.\u003c/p\u003e"},{"header":"CASE REPORT","content":"\u003cp\u003eA 60-year-old Asian man living in the northern Philippines presented with a one week onset of redness in the right eye, severe eye pain (7/10 on the pain scale), tenderness, headache, photophobia, and foreign body sensation. He had a known case of psoriasis for more than 20 years and was maintained on methotrexate and topical medications for psoriasis but with poor compliance. The medical history revealed a previous admission two weeks prior due to COVID-19 infection.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003ePhysical examination revealed multiple lichenified scaly patches on the scalp, neck, and elbows, and erythematous lesions with silvery scales, hypopigmented macules, and papules on the trunk \u003cem\u003e(Fig.\u0026nbsp;1A-B)\u003c/em\u003e. A Boutonniere deformity was noted on the left fifth digit with limited flexion and extension of the distal interphalangeal joints, but not tender on palpation \u003cem\u003e(Fig.C, arrow)\u003c/em\u003e. Examination of all the nails revealed oil spots, nail pits, and nail deformities \u003cem\u003e(Fig.\u0026nbsp;1C-D).\u003c/em\u003e\u003c/p\u003e \u003cp\u003eOphthalmic examination revealed a VA of 20/100\u0026thinsp;+\u0026thinsp;3 in OD, which did not improve on pinhole, and 20/25\u0026thinsp;+\u0026thinsp;2 in OS, which improved to 20/20. The IOP was 12mmHg in both eyes. Biomicroscopy of OD showed conjunctival hyperemia, scleral edema, and ischemia with prominent episcleral and deep scleral vessels in the inferonasal area near the limbus (\u003cem\u003eFigure A\u003c/em\u003e). An adjacent stromal infiltrate with minimal dye uptake was observed \u003cem\u003e(Fig A, arrow)\u003c/em\u003e. Anterior chamber evaluation revealed fine keratic precipitates, +\u0026thinsp;4 cells and flare and an irregular pupil due to posterior synechiae (\u003cem\u003eFigure B\u003c/em\u003e). The phenylephrine test revealed no blanching of the deep episcleral vessels. A lens grading of NO3-NC3 was noted. The left eye was unremarkable. Funduscopy findings in both eyes were normal.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eA working diagnosis of sclerokeratouveitis was made. Gram staining, culture, and KOH of corneal scrapings and eye discharge were negative. Hematology revealed anemia and leukocytosis. Urinalysis revealed an increase in WBCs. Inflammatory markers, ESR and CRP were elevated. Rheumatoid factor, ANA, VDRL, and RPR were all negative. Chest radiography and the Mantoux test results were also negative.\u003c/p\u003e \u003cp\u003eOral prednisone was immediately started at 1 mg/kg/day and gradually decreased after a clinical response. The patient was referred to the Rheumatology service, where the use of biological immunosuppressants was contemplated, but was deferred due to financial constraints and a good clinical response to steroids. The application of topical calcipotriol to skin lesions was prescribed by the Dermatology service.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eWeekly follow-up revealed a gradual decrease in eye pain (3/10), eye redness, and marked improvement in uncorrected visual acuity from 20/100 to 20/30 in the right eye. After three weeks, the anterior chamber was quiet with no synechiae. There was complete resolution of inflammation; however, the area of scleral necrosis appeared thin and translucent, showing a dark choroid underneath it \u003cem\u003e(Fig.\u0026nbsp;3A-B).\u003c/em\u003eThe current plan is to control inflammation, prevent recurrences, and avoid complications of excessive scleral thinning that can lead to perforation or scleral rupture.\u003c/p\u003e"},{"header":"DISCUSSION","content":"\u003cp\u003eNecrotizing scleritis is a rare and severe form with the highest proportion of underlying autoimmune conditions (85.7%), an older age of onset (\u0026gt;\u0026thinsp;45y/o) and the highest rate of complications, in contrast to other types of scleritis [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e, \u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e]. Autoimmune diseases, such as rheumatoid arthritis, granulomatosis with polyangiitis (GPA), relapsing polychondritis, SLE, and PsA, are the most commonly associated entities [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e, \u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e]. On the other hand, infectious causes are less common and are responsible for 5\u0026ndash;10% of cases [\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e]. TB infections, which are endemic in the Philippines, were implicated in 4.2% of the cases in a study in an East Asian population [\u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e]. Other infectious causes include HIV, herpes zoster, syphilis, Pseudomonas aeruginosa, Proteus, and Staphylococcus, with P. aeruginosa being the most common cause of infectious scleritis [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e, \u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e, \u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e]. It has been suggested that the absence of an autoimmune pathology in necrotizing scleritis should alert the physician of a possible infectious etiology [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]. Clinically, infectious scleritis closely resembles autoimmune-associated scleritis and may be impossible to distinguish from one another without histopathologic confirmation. Therefore, it is essential to rule out an infectious etiology before initiating corticosteroids, as this can result in catastrophic complications should an infectious process be present.\u003c/p\u003e \u003cp\u003eOur case presented features most consistent with necrotizing anterior scleritis and keratouveitis in a patient with active psoriasis and PsA. The demographic data of the patient, a man belonging to an older age group, clearly fit the profile. Although we believe that an autoimmune mechanism is at play, the exact event that triggered the inflammatory cascade was not clearly defined, as he reported no history of trauma or surgery and no foci of infection were identified. Notwithstanding, the generally accepted immunopathogenesis is the overproduction of activated matrix metalloproteinases (MMPs) from inflammatory cells and scleral fibroblasts, which causes the breakdown of proteoglycans and collagen and destroys the scleral wall. T-helper cells and macrophages play important roles in inflammatory processes [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e, \u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e]. Furthermore, the highly vascularized nature of episcleral tissue and the choroid beneath the avascular sclera makes it prone to develop vasculitis in autoimmune conditions, where the deposition of immune complexes may lead to more tissue damage. The focal areas of the white sclera observed in our patient represent capillary closure of the episcleral vasculature, which results in infarction and necrosis of the underlying sclera. The combination of severe vasculitis, infarction, necrosis, and choroid exposure causes severe pain and extreme scleral tenderness, consistent with the symptomatology of our patient. Most often adjacent ocular structures, such as the cornea, the ciliary body, and the trabecular meshwork are involved, manifesting as secondary corneal ulceration, peripheral ulcerative keratitis, uveitis, and increased intraocular pressure. These complications were clearly present in our case, except for IOP elevation; however, all were resolved with the initiation of systemic steroids. Although, recurrences have been reported by the Oasis Autoimmune Systemic Inflammatory Infectious Study (OASIS), and most of them were associated with necrotizing scleritis (26.6%) [\u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e]. Therefore, periodic eye monitoring is imperative as part of the long-term management plan for these patients.\u003c/p\u003e \u003cp\u003eTopical NSAIDs are first-line agents but are generally avoided in acute scleritis due to the risk of scleral melt [\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e]. In addition, topical corticosteroids can cause delayed wound healing and perpetuate further scleral thinning. For severe non-infectious scleritis, such as in this case, systemic therapy with oral steroids is the preferred first-line treatment [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e, \u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e]. The use of cyclosporine as a steroid-sparing agent or adjunctive therapy has been reported to be effective but methotrexate remains the first-choice treatment [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e] However, recent developments have highlighted the successful use of biologics as steroid-sparing drugs to selectively target immune mediators in the inflammatory cascade.\u003c/p\u003e \u003cp\u003eOur patient had a recent bout of Covid-19 infection. On presentation, the patient tested negative for RTPCR. However, COVID-19 could have played a role in the exacerbation of psoriasis and its ocular manifestation. Covid-19 disease causes overwhelming inflammation in some patients, also known as cytokine storm, which is more destructive to organs than the disease itself [\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e]. In some cases, necrotizing anterior scleritis was reported in two patients who recovered from coronavirus. Both have no underlying autoimmune disorders. Ocular symptoms appeared 2\u0026ndash;3 weeks after recovery from COVID-19 and were successfully treated with immunosuppressants and biologic agents [\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e]. Although there is no established link that can directly implicate Covid-19 and necrotizing anterior scleritis, it is widely accepted that Covid-19 is a pro-inflammatory condition that could have played a role in the exacerbation of ocular symptoms.\u003c/p\u003e"},{"header":"SUMMARY","content":"\u003cp\u003eNecrotizing anterior scleritis is a rare and severe inflammatory disease, even more rare in the context of psoriasis and PsA. Early recognition and aggressive treatment with systemic oral steroids as first-line agents, together with topical immunomodulators, were effective in controlling inflammation and preventing its sequelae. In addition, a multidisciplinary team of ophthalmologists, rheumatologists, and dermatologists is warranted for the proper long-term management of these cases.\u003c/p\u003e \u003cp\u003eFinancial disclosures: The authors declare that they have no known competing financial interests or personal relationships that could have influenced the work reported in this study.\u003c/p\u003e \u003cp\u003eConsent: The patient consented to the publication and use of all the images in this report.\u003c/p\u003e \u003cp\u003eData Access Statement: All data are contained in the report\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003eFinancial disclosures: The authors declare that they have no known competing financial interests or personal relationships that could have influenced the work reported in this study.\u003c/p\u003e\n\u003cp\u003eConsent: The patient consented to the publication and use of all the images in this report.\u003c/p\u003e\n\u003cp\u003eData Access Statement: All data are contained in the report\u003c/p\u003e\n\u003cp\u003eConflict of interest: None\u003c/p\u003e\n\u003cp\u003eAuthor Contributions: WS collected the data and images, WS and RS wrote the manuscript, and MDP critically revised the manuscript. All the authors approved the final version of the manuscript.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003eConstantin MM, Ciurduc MD, Bucur S, Olteanu R, Ionescu RA, Constantin T, Furtunescu F (2021) Psoriasis beyond the skin: Ophthalmological changes (Review). Exp Ther Med 22(3):981. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.3892/etm.2021.10413\u003c/span\u003e\u003cspan address=\"10.3892/etm.2021.10413\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003eEpub 2021 Jul 12. PMID: 34345263; PMCID: PMC8311223\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eAlgarni A, Almuqrin A, Alarwan A (2017) Association between psoriasis and ocular disorders: A narrative review. 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Med (Kaunas) 58(2):144. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.3390/medicina58020144\u003c/span\u003e\u003cspan address=\"10.3390/medicina58020144\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003ePMID: 35208467; PMCID: PMC8876409\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eFeizi S, Meshksar A, Naderi A, Esfandiari H (2021) Anterior Scleritis Manifesting After Coronavirus Disease 2019: A Report of Two Cases. Cornea 40(9):1204\u0026ndash;1206. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1097/ICO.0000000000002795\u003c/span\u003e\u003cspan address=\"10.1097/ICO.0000000000002795\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003ePMID: 34351874; PMCID: PMC8330625\u003c/span\u003e\u003c/li\u003e\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":true,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"ocular psoriasis, necrotizing scleritis, keratouveitis, psoriatic arthritis, case report","lastPublishedDoi":"10.21203/rs.3.rs-4370909/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-4370909/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003eObjective: To present a rare case of necrotizing anterior scleritis with keratouveitis in a patient with psoriasis and to emphasize the importance of early recognition and aggressive systemic therapy to prevent its sequelae.\u003c/p\u003e\n\u003cp\u003eNecrotizing anterior scleritis is a rare and severely debilitating condition in patients with psoriasis. We present the case of a 60-year-old man with a one-week onset of right eye redness, severe eye pain, and tenderness, accompanied by headache, photophobia, and foreign body sensation. The patient had a history of psoriasis for \u0026gt;20 years and was maintained on methotrexate and topical medications, with poor compliance. He was admitted two weeks prior due to COVID-19 infection. Examination of the right eye showed signs of anterior scleritis and kerato-uveitis. Autoimmune and infection screens yielded negative results. He was administered a regimen of topical cyclosporine BID, atropine TID, and oral prednisone at 1 mg/kg/day that was tapered accordingly after clinical response. Follow-up over the course of three weeks revealed decreased eye pain and improved visual acuity from 20/100 to 20/30 in the right eye. This case of necrotizing sclerotic sclerosis occurred in a setting of long-standing psoriasis and psoriatic arthritis with chronic methotrexate use and various environmental triggers. Early recognition and aggressive treatment with systemic and topical immunomodulators are required to prevent the sequelae of this disease.\u003c/p\u003e","manuscriptTitle":"Necrotizing Anterior Scleritis with kertouveitis in a Patient with Psoriasis: A Case Report","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2024-05-13 15:56:33","doi":"10.21203/rs.3.rs-4370909/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"bc05c76e-8320-4035-91b7-330b78112064","owner":[],"postedDate":"May 13th, 2024","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2025-03-03T09:39:06+00:00","versionOfRecord":[],"versionCreatedAt":"2024-05-13 15:56:33","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-4370909","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-4370909","identity":"rs-4370909","version":["v1"]},"buildId":"qtupq5eGEP_6zYnWcrvyt","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}