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1/49. Vogt-Koyanagi-Harada syndrome after cutaneous injury.

    OBJECTIVE: To describe three patients who developed Vogt-Koyanagi-Harada syndrome (VKH) after cutaneous injury. DESIGN: Retrospective case series. PARTICIPANTS: Three patients seen in the uveitis clinic at Aravind eye Hospital and Postgraduate Institute of ophthalmology, Madurai, india, participated. MAIN OUTCOME MEASURES: The history, evaluation, and management of the three patients were summarized. RESULTS: Three patients developed VKH syndrome shortly after cutaneous injury. In each case, the affected area of skin became vitiliginous on healing and simultaneous with the onset of ocular symptoms. One patient developed additional ectopic areas of vitiligo. All three patients developed chronic, bilateral, diffuse uveitis, one associated with an exudative retinal detachment and two with Dalen-Fuchs-like nodules. Well-recognized complications of VKH syndrome that occurred in the authors' patients included geographic atrophy of the retinal pigment epithelium (3 of 3), cataract (3 of 3), and glaucoma (1 of 3). Ocular inflammation was well controlled in each patient with local or systemic corticosteroids or both. In one patient, the area of vitiligo showed increased pigmentation in response to systemic corticosteroid treatment. CONCLUSIONS: Vogt-Koyanagi-Harada syndrome may follow cutaneous injury, supporting the notion that this disorder may result from systemic sensitization to shared melanocytic antigens.
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ranking = 1
keywords = detachment, retinal detachment, retinal pigment, pigment
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2/49. Vogt-Koyanagi-Harada syndrome in an 11-year-old Boy.

    An 11-year-old boy complained of headache, slight fever and decreased visual acuity. Intracameral cells and serous retinal detachment were found in both eyes. Pleocytosis was seen in the cerebrospinal fluid. Bilateral uveitis diminished rapidly in response to corticosteroid treatment. Depigmentation of the fundi developed several months later. We believe that Vogt-Koyanagi-Harada syndrome in a child, as demonstrated in our patient, may be uncommon.
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ranking = 0.86210952027308
keywords = detachment, retinal detachment, pigment
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3/49. Extensive chorioretinal atrophy in Vogt-Koyanagi-Harada disease.

    PURPOSE: To report extensive chorioretinal atrophy during the long-term course of Vogt-Koyanagi-Harada (VKH) disease not treated properly in the initial phase. CASES: Four patients with VKH disease were examined more than 10 years after onset of the disease. OBSERVATIONS: They presented initially with classic features of VKH disease, except 1 patient who had developed bilateral, acute angle-closure glaucoma as the initial sign. Two patients received systemic corticosteroid therapy at the acute phase of the disease. During the follow-up of 13-34 years subsequent to onset, these patients had chronic recurrent anterior uveitis with apparently stable depigmented fundus. Eventually, they developed diffuse, extensive chorioretinal atrophy that resulted in severe visual loss. One patient had an unusual familial occurrence of the disease. CONCLUSIONS: Failure to prescribe proper corticosteroid therapy in the initial phase of VKH disease may lead to chronic recurrent uveitis. Long-standing uveitic reactions may eventually result in severe visual loss due to extensive chorioretinal degeneration.
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ranking = 0.00921798287458
keywords = pigment
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4/49. Quantitative evaluation of "sunset glow" fundus in Vogt-Koyanagi-Harada disease.

    PURPOSE: To evaluate the color of the fundus quantitatively, especially the "sunset glow" fundus, in patients with Vogt-Koyanagi-Harada (VKH) disease. methods: The fundus of 39 patients (13 men and 26 women) who were diagnosed with VKH disease were photographed. The photographs were scanned by a film scanner and the amount of red, green, and blue pixels making up the image was determined by image analyzing software. A "sunset glow" index, the ratio of the number of red pixels to the total number of pixels, was determined for all patients as well as 31 normal controls. RESULTS: In comparison to the controls, the "sunset glow" indices at 3 months after onset of the "sunset glow" fundus showed significant increases in VKH patients. Six months after onset, the "sunset glow" indices showed further significant increases and continued to increase during the course of the disease. CONCLUSIONS: The depigmentary changes can be found earlier by using our method rather than ophthalmoscopy. Because our method is more sensitive for slight depigmentary changes, the pigmentary changes in the fundus could be found in all the VKH patients. This quantitative evaluation of the fundus makes a correct diagnosis possible even in patients who do not demonstrate the "sunset glow" fundus by normal ophthalmoscopic examinations.
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ranking = 0.02765394862374
keywords = pigment
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5/49. Histopathologic findings in the alopecia associated with Vogt-Koyanagi-Harada disease.

    BACKGROUND: Vogt-Koyanagi-Harada (VKH) disease is a rare autoimmune disease primarily involving pigmented structures. The commonly associated alopecia usually precedes leukodermic changes seen in the hair and skin. OBJECTIVE: We present a 46-year-old oriental woman with VKH disease who developed diffuse alopecia followed by the regrowth of nonpigmented hairs. The biopsy specimens showed a peribulbar mononuclear infiltrate with increased telogen/catagen:anagen follicles. The most prominent additional histologic finding was melanin pigment release from the matrix into the dermal papillae, fibrous tracks, and surrounding perifollicular sheaths. The peribulbar mononuclear cells showed diffuse immunohistochemical staining of approximately 80 to 90% of the cells for CD3, CD45RO. More than 50% of the mononuclear cells showed positive staining for CD4, whereas approximately 10 to 20% showed staining for TIA with TIA cells within the follicular epithelium. CONCLUSION: Although the histologic features seen in the alopecia associated with VKH are consistent with alopecia areata, the prominent pigment release does suggest that the prime target are the melanocytes and that keratinocytes may be secondarily involved.
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ranking = 0.03687193149832
keywords = pigment
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6/49. A case of insulin dependent diabetes mellitus following systemic treatment for Vogt-Koyanagi-Harada syndrome.

    A 29-year-old man developed acute visual impairment, cough, and headache. Both eyes showed serous retinal detachment in the posterior fundus. fluorescein angiography showed subretinal pooling of fluorescein in the late phase. A diagnosis of Vogt-Koyanagi-Harada (VKH) syndrome was made based on clinical features. Treatment with systemic corticosteroids resulted in improvement of uveitis and both eyes showed "sunset glow" fundus 11 months later. insulin-dependent diabetes mellitus (IDDM) developed 13 months later (3 months after systemic corticosteroid therapy). Despite treatment with insulin, glycemic control was poor. Human leukocyte antigen (HLA) typing showed HLADR9 and DQB 1*0303 related to IDDM. We postulated that treatment with corticosteroids precipitated IDDM, a yet unknown common autoimmune mechanism might have caused IDDM and VKH, or both conditions occurred coincidentally.
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ranking = 0.8528915373985
keywords = detachment, retinal detachment
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7/49. Inflammatory vitiligo in Vogt-Koyanagi-Harada disease.

    Vogt-Koyanagi-Harada disease is a rare disease characterized by uveitis, meningitis, dysacusis, alopecia, poliosis, and vitiligo. We describe a 48-year-old patient with Vogt-Koyanagi-Harada disease associated with thin inflammatory raised erythema and plaque-type inflammatory erythema superimposed on vitiligo. Interestingly, inflammatory raised erythema was separated from the perfect vitiligo, and the incomplete vitiligo lay between them initially. Thereafter, incomplete vitiligo became completely depigmented with diminution of inflammatory raised erythema. This is the second case of vitiligo with inflammatory raised borders associated with Vogt-Koyanagi-Harada disease. Our results of immunohistochemical and electron microscopic studies suggested the involvement of T-cell-mediated cytotoxicity and apoptosis in the development of skin lesions.
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ranking = 0.00921798287458
keywords = pigment
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8/49. Depigmented atrophic lesions in sunset glow fundi of Vogt-Koyanagi-Harada disease.

    PURPOSE: Although the depigmented, small, round to oval lesions seen in the sunset glow fundi of Vogt-Koyanagi-Harada disease are considered to represent Dalen-Fuchs nodules, there is no histopathologic evidence to support such a consideration. An attempt is made herein to clarify the nature of the atrophic lesions and distinguish them from Dalen-Fuchs nodules seen in eyes with Vogt-Koyanagi-Harada disease. methods: Eyes from five individuals with clinical diagnoses of Vogt-Koyanagi-Harada disease were subjected to histopathologic examination. The retinal pigment epithelial changes from early active to convalescent and late chronic recurrent stages were evaluated. Particular attention was paid to Dalen-Fuchs nodules, depigmented lesions in the sunset glow fundi, and hyperpigmentation of the chronic recurrent stage. RESULTS: Eyes of two individuals, one in the active stage of Vogt-Koyanagi-Harada disease and the other in the convalescent stage, showed the presence of Dalen-Fuchs nodules. The depigmented small retinal pigment epithelial lesions were seen in two individuals, both of whom exhibited the sunset glow fundus of the convalescent stage. The retinal pigment epithelial lesions represented damage or disappearance of retinal pigment epithelial cells, and the sunset glow fundus appearance was from the loss of choroidal melanocytes. The heavy pigmentation seen in fundi with the chronic recurrent stage was the result of the proliferation of retinal pigment epithelial cells. CONCLUSION: The Dalen-Fuchs nodule is a specific histologic change observed at the level of retinal pigment epithelium in patients with Vogt-Koyanagi-Harada disease. There is no histologic confirmation that the depigmented small atrophic lesions seen in the sunset glow fundi of Vogt-Koyanagi-Harada disease are Dalen-Fuchs nodules. The depigmented lesions represent localized damage or disappearance of retinal pigment epithelial cells.
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ranking = 1.0666311697088
keywords = retinal pigment, pigment
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9/49. Photodynamic therapy with verteporfin for subfoveal choroidal neovascularization in Vogt-Koyanagi-Harada syndrome.

    PURPOSE: To assess the role of photodynamic therapy using verteporfin in the treatment of subfoveal choroidal neovascularization in Vogt-Koyanagi-Harada syndrome. DESIGN: Interventional case report. methods: A 9-year-old patient with subfoveal choroidal neovascularization received a single photodynamic therapy with verteporfin session (one eye) and was prospectively followed with fluorescein angiography. RESULTS: A complete regression of the lesion was achieved within 1 week after treatment. visual acuity improved from 20/800 to 20/320 by 6 months of follow-up. fluorescein angiography disclosed unexpected retinal pigment epithelium alteration within the treatment area. CONCLUSION: Although regression of the choroidal neovascularization occurred, unpredicted findings involving normal retina in the vicinity of the lesion suggest that further studies are required to assess the clinical value of this treatment for subfoveal choroidal neovascularization in Vogt-Koyanagi-Harada syndrome.
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ranking = 0.13789047972692
keywords = retinal pigment, pigment
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10/49. Ultrasound biomicroscopic study of ciliary body changes in the post-treatment phase of Vogt-Koyanagi-Harada disease.

    AIMS: To investigate the usefulness of ultrasound biomicroscopy for evaluating changes in the ciliary body in patients with Vogt-Koyanagi-Harada disease. methods: Ultrasound biomicroscopy was used to evaluate 14 eyes of seven patients diagnosed with Vogt-Koyanagi-Harada disease. Cross sectional images of the ciliary body and thickness of the pars plana 3.0 mm posterior to the scleral spur were examined. Predicted thickness of the pars plana was obtained by multiple linear regression analysis of thickness in the acute phase and in the remission phase. RESULTS: In the active phase, the cross sectional images showed a shallow anterior chamber in eight of the 14 eyes, ciliochoroidal detachment in five eyes, and a thickened ciliary body in all 14 eyes. Internal reflectivity of the ciliary stroma was low, with ciliary processes being unclear in 13 eyes. One month after steroid treatment, slit lamp examination findings were normal in 14 eyes. 10 eyes of five patients were examined by ultrasound biomicroscopy at this stage. Ciliochoroidal detachment was no longer seen in any eye. Internal reflection of the ciliary stroma became relatively homogeneous, and the ciliary processes were seen, though not clearly. However, the pars plana remained thickened. The actual thickness was greater at 1 month after steroid treatment than the predicted thickness for the remission phase. In the remission phase, the internal reflection was homogeneous and the ciliary processes were delineated clearly in all 14 eyes. CONCLUSION: Objective, quantitative evaluation of the ciliary body is possible with ultrasound biomicroscopy during the course of Vogt-Koyanagi-Harada disease. Ultrasound biomicroscopy is useful in determining disease activity in the anterior segment and in monitoring the clinical course, and it may improve evaluation of the efficacy of treatment.
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ranking = 0.93118145477842
keywords = detachment
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