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      Reversible alopecia in Vogt-Koyanagi-Harada disease and sympathetic ophthalmia

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          Abstract

          Background

          Vogt-Koyanagi-Harada (VKH) disease and sympathetic ophthalmia (SO) are both autoimmune disorders targeting melanin-bearing cells, even though their etiologies are different. Both shared many ocular and systemic manifestations, including integumentary findings. Most of the literature focused on the ocular manifestations and related treatment. Alopecia was seldom mentioned.

          Findings

          We report one case of VKH disease and one case of SO. Both developed severe alopecia and early sunset glow fundus, which are probably due to incomplete treatment. Fortunately, the alopecia improved soon after systemic steroid treatment.

          Conclusions

          Early and complete treatments are important in the management of VKH or SO and prevent integumentary manifestation. Alopecia can be reversible after steroid treatment in time.

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          Most cited references10

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          Update on Sympathetic Ophthalmia

          Sympathetic ophthalmia (SO) is a bilateral diffuse granulomatous intraocular inflammation that occurs in most cases within days or months after surgery or penetrating trauma to one eye. The incidence of SO ranges from 0.2 to 0.5% after penetrating ocular injuries and 0.01% after intraocular surgery. Vitreoretinal surgery and cyclodestructive procedures are considered risk factors. The time from ocular injury to onset of SO varies greatly, ranging from a few days to decades, with 80% of the cases occurring within 3 months after injury to the exciting eye and 90% within 1 year. The diagnosis is based on clinical findings rather than on serological testing or pathological studies. It presents as a bilateral diffuse uveitis. Patients report an insidious onset of blurry vision, pain, epiphora, and photophobia in the sympathizing, non-injured eye. Classically this is accompanied by conjunctival injection and a granulomatous anterior chamber reaction with mutton-fat keratic precipitates (KPs) on the corneal endothelium. In the posterior segment, the extent of inflammation can vary. Systemic corticosteroids are the first line therapy for SO. If patients are non-responsive to steroid therapy or have clinically significant side effects, cyclosporine, azathioprine or other immunosuppressive agents can be used for long-term immunomodulatory therapy.
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            Evaluation of the effect on outcomes of the route of administration of corticosteroids in acute Vogt-Koyanagi-Harada disease.

            To compare the effect on outcomes of the route of administration of corticosteroids in acute Vogt-Koyanagi-Harada disease. Retrospective comparative interventional case series. Nine international uveitis specialty clinics. Forty-eight patients presenting over a three-year period to a study center with acute Vogt-Koyanagi-Harada disease. Initial treatment with corticosteroid either orally (Oral only group) or intravenously followed by an oral taper (IV+Oral group). Change in visual acuity with treatment; development of ocular complications, including visually significant cataract, choroidal neovascularization, subretinal fibrosis, fundus pigment migration, nummular hypopigmented lesions, and diffuse fundus depigmentation; use of immunosuppressive therapy. The Oral only group comprised 15 patients (31%) and the IV+Oral group 33 patients (69%). Median follow-up was 15 months. There was no difference in duration of follow-up between groups (P = .234). There was no difference in the change in visual acuity between groups, adjusting for initial visual acuity (P = .402). There were no differences in the rates of development of visually significant cataract, fundus pigmentary changes, or in the rate of use of subsequent immunosuppressive therapy between treatment groups. No patients developed choroidal neovascularization or subretinal fibrosis over the study period. Route of administration of corticosteroid had no detectable effect on change in visual acuity nor on the development of visually significant complications over the study period. Prospective trials are necessary to address speed of resolution and definitively answer outcome questions.
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              Mechanisms of inflammatory response in sympathetic ophthalmia and VKH syndrome.

              Although the inciting events in the pathogenesis of sympathetic ophthalmia and Vogt-Koyanagi-Harada (VKH) syndrome are different, these two forms of bilateral, granulomatous uveitis share several clinical, histopathological and immunohistochemical features, including their association with HLA types and in their in vitro T-cell response to retinal antigens. These clinical and immunopathological features indicate that there is an underlying T-cell mediated autoimmunity to uveal/retinal antigens in the development of these forms of uveitis. Both forms exhibit preservation of the choriocapillaris and retina despite extensive inflammatory cell infiltration in the choroid. Recent experimental studies suggest that this preservation of choriocapillaris could be the result of anti-inflammatory products secreted by the retinal pigment epithelium, including transforming growth factor-beta and a novel protein called retinal pigment epithelial protective protein that is known to suppress the phagocyte generation of superoxide. Such suppression of the oxidant release in the choroidal inflammation could help protect the uvea from necrotic change and preserve the choriocapillaris from inflammatory cell infiltration.
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                Author and article information

                Contributors
                Journal
                J Ophthalmic Inflamm Infect
                J Ophthalmic Inflamm Infect
                Journal of Ophthalmic Inflammation and Infection
                Springer
                1869-5760
                2013
                15 March 2013
                : 3
                : 41
                Affiliations
                [1 ]Department of Ophthalmology, Kaohsiung Veterans General Hospital, 386, Ta-Chung 1st Road, Kaohsiung, 813, Taiwan
                [2 ]School of Medicine, National Yang-Ming University, Taipei, 112, Taiwan
                Article
                1869-5760-3-41
                10.1186/1869-5760-3-41
                3620517
                23514340
                5f5c4424-0f0d-490f-bc6e-243f0543f2a1
                Copyright ©2013 Chuang et al.; licensee Springer.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 25 September 2012
                : 25 January 2013
                Categories
                Brief Report

                Ophthalmology & Optometry
                Ophthalmology & Optometry

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