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Purpose: To share our initial experience with the innovative use of topical erythropoietin for the treatment of necrotizing scleritis manifesting immediately after pterygium excision surgery., Methods: This study enrolled 3 patients who developed necrotizing scleritis immediately after undergoing pterygium excision. All cases with pterygium were primary, and topical mitomycin C and conjunctival autografts were used at the time of surgery. Noninvasive therapy that included ophthalmic lubricants and topical and systemic corticosteroids failed to improve the avascular scleral lesions. The patients were prescribed erythropoietin-containing drops (3000 U/mL) every 6 hours in addition to topical antibiotics and lubricant. The effect of topical erythropoietin on the healing process of avascular scleral lesions was investigated, and its ocular and systemic side effects were evaluated., Results: The mean age of the participants was 69.0 ± 14.8 years, and 2 of the 3 eyes belonged to male subjects. The time between pterygium surgery and presentation to our clinic was 33.0 ± 14.7 days. There were no infectious causes or underlying systemic diseases in any of the cases. After treatment with topical erythropoietin for an average of 34.3 ± 20.3 days, the lesions were completely vascularized in all 3 eyes without any ocular or systemic adverse effects. The patients were followed up for an average of 126 ± 94 days after discontinuation of erythropoietin. There was no evidence of recurrence during the last examination in any of the eyes., Conclusions: Topical erythropoietin might be a safe and an effective method for treating cases of necrotizing scleritis that manifests immediately after pterygium surgery., Competing Interests: The authors have no funding or conflicts of interest to disclose., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose: To correlate the clinical course of sympathetic ophthalmia with the histological and immunohistochemical characteristics of the enucleated inciting eye., Methods: A consecutive case series with baseline clinical features and subsequent histopathologic findings., Results: Evaluation of the 16 enucleated inciting eyes (blind and painful) disclosed that 9 of the 16 had typical histology, fulfilling the criteria for sympathetic ophthalmia of diffuse granulomatous inflammation. Among the 16, 11 sustained previous penetrating trauma, 4 underwent previous eye surgery, and 1 patient presented with an unknown etiology. Patients with atypical histology (7 of 7) were taking corticosteroids at the time of enucleation. Only 2 of 9 patients with typical histology were taking corticosteroids at the time of enucleation. At 6 months after enucleation of the inciting eye, 4 of the 7 patients with atypical histology had a visual acuity of ≥20/40 compared with 8 of 8 patients (100%) with typical histology. On a 4-point scale (0-3+), the choroidal infiltrate of the 9 histopathologically typical eyes showed an average of 2.5+ CD68 (macrophages), 2.5+ CD20 (B cells), and 1.5+ CD3 (T cells)., Conclusion: Histopathologic findings had minimal correlation with the clinical course of sympathetic ophthalmia. Corticosteroid treatment before enucleation may influence the pathologic confirmation of sympathetic ophthalmia. The predominance of B lymphocytes and macrophages over T lymphocytes may represent different stages of the disease process.

A 57-year-old man presented to the ophthalmology clinic with a red right eye. He denied pain, diplopia, tearing, and blurred vision. His medical history included asymptomatic annular plaques on the trunk and extremities for at least a decade. Ophthalmological examination revealed a necrotizing scleritis of the right eye. Examination of the skin demonstrated variable sized annular plaques with central atrophy, some with prominent indurated border and yellow discoloration. No periorbital lesions were present. The ocular lesion rapidly progressed and areas of scleral melting developed in the right eye, which eventually required a scleral patch graft. The left eye also developed necrotizing scleritis with areas of scleral melting. Two sets of skin biopsies were performed a few weeks apart. An initial set of skin punch biopsies revealed extensive palisading granulomatous inflammation throughout the dermis, extending into the subcutis. The accompanying perivascular mononuclear infiltrate contained the collections of plasma cells. Scattered multinucleated giant cells were noted. The possibility of necrobiosis lipoidica diabeticorum was suggested. Subsequent skin biopsies showed more prominent and extensive necrobiosis, raising the possibility of necrobiotic xanthogranuloma. Protein electrophoresis was performed, which revealed an IgG λ monoclonal protein.

Objectives: To demonstrate an unusual case of necrotizing scleritis in ulcerative colitis successfully managed by a combination of a Prokera implant and processed pericardium., Methods: A case report of a novel approach to treat scleral melts that may be seen in several clinical situations., Results: The dual use of amniotic membrane material (Prokera) with processed tuttoplast for necrotizing scleritis salvaged the eye without the need for enucleation while also preserving most of the vision., Conclusions: Consideration of the combined method discussed in this article should be considered in the treatment of severe scleromalacia seen in inflammatory cases of the eye.

Purpose: A case of keratectasia is reported as a severe presentation of surgically induced necrotizing sclerokeratitis (SINS)., Methods: A 72-year-old white woman had a painful, raised lesion on the superior cornea of her right eye 3 years after uncomplicated extracapsular cataract extraction with intraocular lens implantation. Examination showed a large ectatic area of the superior cornea with inflamed sclera adjacent to the surgical wound, which was diagnosed as SINS., Results: No underlying systemic autoimmune condition or vasculitis was identified on investigation. Progressive painful keratectasia necessitated enucleation, which confirmed on histopathologic examination features of chronic nodular episcleritis and nongranulomatous scleritis with evidence of keratitis and fibrovascular scarring., Conclusion: The predominant inflammatory response in the cornea represents surgically induced necrotizing keratoscleritis (SINK) as a new variant presentation of SINS. Oral corticosteroids and immunosuppressive agents should not be delayed to prevent progressive tissue destruction and poor outcome.

Purpose: To describe a case of infectious scleritis caused by methicillin-resistant Staphylococcus aureus (MRSA) after pars plana vitrectomy despite treatment with topical fourth-generation fluoroquinolones., Methods: Single interventional case report., Results: A 72-year-old man presented with scleral thinning and subconjunctival abscess 1 month after pars plana vitrectomy. Microbiological studies including cultures and antibiotic sensitivity were performed. Culture of the scleral abscess showed growth of MRSA resistant to fourth-generation fluoroquinolones and sensitive to vancomycin and erythromycin. Treatment with a combination of topical vancomycin and systemic erythromycin for 6 weeks resolved the infectious scleritis., Conclusions: MRSA can be a cause of infectious scleritis after ocular surgery without a clear source despite surgical prophylaxis with topical fourth-generation fluoroquinolones.

Purpose: This documents the evaluation and management of a patient with an unusual destructive external ocular disorder in the context of progressively disfiguring dermatitis., Methods: Observational case report including ophthalmologic examination with conjunctival and corneal scraping, radiologic studies of the sinuses and chest, skin, conjunctival, and scleral biopsies, and serologic studies., Results: Biopsies, cultures, and serologic testing led to the conclusion that the patient had pyoderma gangrenosum. Therapy with dapsone resulted in preservation of vision and resolution of the oculocutaneous inflammation., Conclusions: Pyoderma gangrenosum is a rare autoimmune disorder that can affect the eye. Biopsy of affected tissue is the key to diagnosis. Immunomodulating therapy can be a useful adjunct to the traditional steroid therapy.

Purpose: To describe the use of a tarsoconjunctival pedicle flap for the repair of a large scleral melt in a patient with rheumatoid arthritis., Methods: Case report and literature review., Results: A 36-year-old woman with a history of rheumatoid arthritis presented to the Rocky Mountain Lions Eye Institute with a large scleral melt in the right eye. Ophthalmic examination revealed a visual acuity of 20/400 and a superior scleral melt measuring 15 x 30 mm. After instituting systemic immunosuppression, a tarsoconjunctival pedicle was used to provide tectonic support to the globe. At her 3-month postoperative visit, her vision improved to 20/30, and the scleral melt was completely epithelialized., Conclusions: A tarsoconjunctival pedicle flap provides an excellent alternative to help restore scleral integrity in patients with severe autoimmune necrotizing scleritis.

In patients with rheumatoid arthritis (RA), necrotizing scleritis, an ocular manifestation of a systemic vasculitic process, is associated with significant ocular morbidity and high mortality. We present a 60-year-old man with RA who developed necrotizing scleritis and peripheral ulcerative keratitis. The scleritis was refractory to local measures, systemic corticosteroids, and cyclophosphamide but responded rapidly to infliximab. Our case illustrates that biologic agents may be considered in refractory cases of sight- and life-threatening scleritis.

Purpose: To review the efficacy of Etanercept as an alternate therapy for treatment of necrotizing anterior scleritis and sterile corneal ulceration unresponsive to traditional therapies., Methods: A retrospective review of 10 patients treated with Etanercept for vision-threatening scleritis and sterile corneal ulceration., Results: Etanercept alone or in combination with other immunosuppressive therapies controlled inflammation, arrested tissue ulceration, and in many cases permitted tapering or cessation of toxic immunosuppressive therapies. No complications or systemic toxicity were observed with Etanercept use., Conclusion: Etanercept is an effective treatment for scleritis and sterile corneal ulceration and has a favorable benefit-to-risk ratio. It may be considered for therapy of progressive disease or cases that are unresponsive to traditional therapies.

Purpose: To report a case of surgically induced necrotizing scleritis (SINS) after pterygium excision and conjunctival autograft., Methods: A 55-year-old man presented 2 weeks after excision of primary pterygium with conjunctival autograft in the right eye with severe pain in that eye. He had undergone cataract surgery in that eye 8 months before. The graft was pale and white. The underlying sclera was white and avascular. There was marked inflammation adjacent to the site of surgery and graft. A scraping from the graft surface revealed no organisms in smears, and culture revealed no growth. The conjunctival graft was removed. Amniotic membrane transplantation was performed. After surgery, the amniotic membrane was avascular and pale. Systemic steroid therapy was initiated., Results: Three days after initiation of systemic steroid therapy, the graft became vascularized. Over the next 10 days, the eye quietened, conjunctival and scleral inflammation lessened, and the graft was well accepted., Conclusions: Surgically induced necrotizing scleritis is a rare complication of pterygium excision with conjunctival autograft. A pale graft with underlying avascular sclera and surrounding inflammation should arouse suspicion of this complication.

Purpose: To perform a clinical, laboratory and pathologic evaluation in patients who had developed a postsurgical necrotizing sclerocorneal ulceration to detect a serious associated autoimmune disorder and to treat the ocular disease early., Methods: Nine patients with postsurgical necrotizing sclerocorneal ulceration after uneventful cataract extraction were studied by means of immunohistochemical techniques on conjunctival resections, immunologic serologic studies, and rheumatologic evaluation. Nine healthy subjects who underwent uneventful cataract surgery were used as controls., Results: The pathologic studies showed a local immunoglobulin M (IgM) and IgG deposition, increased human leukocyte antigen (HLA-DR) expression, and a significant T-helper cell participation in conjunctival biopsies in the most severe ulcerations, which were detected in four patients with underlying autoimmune systemic disorder (rheumatoid arthritis, 45%) and only a macrophagic infiltration in the mildest ulcers in patients (55%) without immune disorders. Serologic features included high titers of rheumatoid factor in the four (45%) patients with rheumatoid arthritis, nonspecific serologic immune alteration in three (33%) patients, and were unremarkable in two (22%) patients. The medical and immunologic evaluations were negative in the control cases. Topically administered cyclosporin A healed the ocular disease., Conclusion: A surgically induced local autoimmune reaction could occur in the incision area in patients with systemic vasculitic disease. There was no underlying systemic disorder in the mildest ulcers, and these ulcers could be due to a defect in the surgical technique. Our results suggest the need for a detailed systemic evaluation in patients with severe postsurgical necrotizing ulceration. Early diagnosis and aggressive medical treatment of the ocular disorder improves the visual outcome.

We present a case of a patient with scleritis and the fibromyalgia syndrome. Scleritis and the fibromyalgia syndrome are both thought to be caused by an immunological process. This is the first report of a possible association of scleritis and the fibromyalgia syndrome. Additional cases might suggest that the fibromyalgia syndrome be added to the list of etiologies of scleritis.

The onset of necrotizing scleritis (NS) and peripheral ulcerative keratitis (PUK) in the clinical course of rheumatoid arthritis (RA) may reflect the presence of systemic, potentially lethal vasculitis. In an effort to better characterize this subset of patients with severe RA-associated corneal and/or scleral inflammation and to analyze the efficacy of therapy, we reviewed our experience in the medical and surgical management of 16 tertiary referral cases (25 eyes) unresponsive to aggressive conventional therapy with topical and systemic steroids as well as with systemic nonsteroidal drugs. Cytotoxic immunosuppressive therapy was instituted in all patients with NS and/or PUK. Cyclophosphamide and methotrexate were the most successful agents used. Cytotoxic immunosuppressive drugs in conjunction with early aggressive surgical treatment halted the relentlessly progressive inflammation and preserved the integrity of the globe in 92% of eyes. Visual acuity could be stabilized or improved in 83% of patients with NS and in 68% of patients with PUK.

Three patients with corneal or scleral defects were treated with Gore-Tex grafting, including one case of corneal ulcer, one case of Mooren's ulcer, and one case of necrotizing scleritis. These eyes remained inflamed after Gore-Tex grafting. The interval between grafting and removal ranged from 2 months to 1 year. Pathologically, none of these grafts showed evident epithelial or fibrous tissue ingrowth of the Gore-Tex graft. Fungal inhabitation was noted in one graft, and fungal endophthalmitis developed 5 months after graft removal and penetrating keratoplasty. Although Gore-Tex offers some advantages, it has disadvantages when it is used without adequate conjunctival covering, including poor epithelialization, poor adhesion between graft and surrounding tissue, and potential infectious route.