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1/11. 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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2/11. 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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3/11. Vogt-Koyanagi-Harada syndrome associated with cutaneous malignant melanoma: an 11-year follow-up.

    PURPOSE: To report a case of Vogt-Koyanagi-Harada (VKH) syndrome associated with cutaneous pigmented malignant melanoma (MM) and non-pigmented nodular metastasis after a 10-year recurrence-free interval. methods: Case report with long-term follow-up of 11 years. Ocular examinations included best-corrected visual acuity (ETDRS charts), fundus photography, fluorescein angiography, and computer-assisted perimetry. In addition, human leukocyte antigen (HLA) typing was performed. RESULTS: A 48-year-old white female patient developed VKH disease 5 years after she had undergone surgical treatment of a superficial spreading melanoma on her back in 1991. The first symptoms were diffuse alopecia followed by growth of non-pigmented hair after 8 months. In our clinic, she presented 18 months later with cells and opacification in the vitreous, a macular and optic disc edema and atrophy of the retinal pigment epithelium (RPE). The anterior segment showed endothelial precipitates of the cornea, and a progressive secondary cataract. fluorescein angiography detected a bilateral edema of the macula and the optic disc, and focal leakage in the RPE level. During follow-up the patient developed more cutaneous signs, such as vitiligo-like depigmentation and poliosis. A non-pigmented lymph node MM metastasis was diagnosed after a 10-year disease-free interval. HLA typing was positive for HLA-A*01, HLA-A*24, HLA-B*08, HLA-B*39, HLA-DRB1*03, and HLADRB1*11. CONCLUSION: Our findings suggest that the described ocular findings of VKH disease may represent a component of a syndrome consisting also of melanoma-associated hypopigmentation. Within the framework of current concepts of immunity in patients with MM and VKH, the long recurrence-free interval might support the hypothesis of an autoimmune or hypersensitivity process against melanocytes. The use of immunosuppressive therapy in the treatment of VKH and its potential influence on the development of metastatic disease should be carefully reconsidered.
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4/11. Vogt-Koyanagi-Harada syndrome following injury-induced progressive vitiligo.

    We report a case of Vogt-Koyanagi-Harada (VKH), syndrome wherein the patient developed ocular symptoms following injury-induced progressive vitiligo with immunologic evidence from the skin biopsy specimen of the vitiligo. This case supports the hypothesis that the cell-mediated immune process against a common melanocyte antigen plays an important role in the development and progression of the disease.
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5/11. Class II major histocompatibility complex on melanocytes of Vogt-Koyanagi-Harada disease.

    The eyes obtained from two Japanese autopsy cases of patients with Vogt-Koyanagi-Harada disease were immunohistochemically examined. Both patients, a 63-year-old woman and a 68-year-old man, were clinically and histologically diagnosed as having Vogt-Koyanagi-Harada disease. Immunohistochemically, the choroidal infiltrate was composed predominantly of T lymphocytes with a larger proportion of helper/inducer T cells than suppressor/cytotoxic T cells and it also included activated lymphocytes expressing CD26 and CD25 antigens. Class II major histocompatibility complex was expressed in the choroidal melanocytes as well as in the endothelium of the choriocapillaris. Depositions of complement, however, were focally noticed in the choroid. Our results indicate that the cell-mediated immune process plays an important role in the development and progression of Vogt-Koyanagi-Harada disease, while choroidal melanocytes appear to play a pathogenic role in this disease.
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6/11. Clinical studies of Vogt-Koyanagi-Harada's disease at the National eye Institute, NIH, USA.

    We have, in the recent past, seen 46 Vogt-Koyanagi-Harada's disease patients at the National eye Institute. Most of these patients have been women, and a high percentage were Black-American or Hispanic. However, an interesting observation has been American Indian antecedents for a large number of these patients, whether they considered themselves Black or Caucasian. The visual acuities tended to be at the extremes of the vision chart. patients with this disorder appear to have circulating autoantibodies to the retinal photoreceptor region, and these autoantibodies do not appear to be directed against the retinal S-antigen.
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7/11. Simultaneous onset of Vogt-Koyanagi-Harada syndrome in monozygotic twins.

    PURPOSE/methods: We examined monozygotic twins of Vietnamese ancestry in whom the Vogt-Koyanagi-Harada syndrome developed. RESULTS/CONCLUSIONS: Both patients demonstrated typical clinical findings of the Vogt-Koyanagi-Harada syndrome, and HLA typing disclosed HLA-DR4, an antigen that is present in a disproportionate number of patients with the Vogt-Koyanagi-Harada syndrome. Although familial cases of the Vogt-Koyanagi-Harada syndrome are rare, associations with hla antigens may indicate that genetic factors play a role in this disease.
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8/11. Vogt-Koyanagi-Harada syndrome.

    The case history is presented of a 40-year-old White women with the Vogt-Koyanagi-Harada syndrome, who was successfully treated with a combination of low-dose prednisolone and cyclosporine. A. Cellular and humoral hypersensitivity to uveal and retinal antigens were demonstrated in the patient.
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9/11. Immunocytologic findings in a case of Vogt-Koyanagi-Harada syndrome.

    BACKGROUND: Vogt-Koyanagi-Harada (VKH) syndrome is a form of chronic panuveitis that often has a poor long-term visual outcome. To the authors' knowledge, there have been only two previous reports describing immunocytologic findings in the eyes of patients with VKH syndrome, all studied in relatively late stages of the disease. methods: The authors performed immunocytologic studies on the enucleated eyes of a patient with active VKH syndrome, using monoclonal antibodies to mononuclear subsets, major histocompatibility antigens, and viral and treponemal agents, to better understand the pathophysiologic mechanisms underlying the disease. Emulsified chorioretinal tissue also was processed for viral isolation in tissue culture and for treponema pallidum by rabbit inoculation studies. RESULTS: The dense uveal infiltrates in active VKH syndrome are composed predominantly of T lymphocytes and HLA-DR macrophages. Scattered T cells and plasma cells were present in the retina. In addition, nondendritic-appearing CD1 (Leu-6) positive cells were localized in the choroid, in close proximity to choroidal melanocytes. No microbial agents were identified. CONCLUSION: Whereas the etiology of the VKH syndrome remains an enigma, the authors' immunocytologic findings are consistent with a T-cell-mediated disorder. In demonstrating CD1 (Leu-6) positive cells in the infiltrates, the authors propose a potential role for these cells in the etiology of VKH syndrome.
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10/11. Clinical and immunogenetic investigation of a Laotian patient with Vogt-Koyanagi-Harada's disease.

    Vogt-Koyanagi-Harada's (VKH) disease has been known to be associated with HLA class II antigen, HLA-DR4 and -DR53. Recent recombinant dna technical advances have allowed us to investigate the genetic polymorphism of hla antigens at the molecular level. By use of the polymerase chain reaction restriction fragment length polymorphism (PCR-RFLP) technique we have found that VKH disease is closely associated with HLA-DRB1*04 (DRB1*0405/ DRB1*0410) and DQB1*04 (DQB1*0401/DQB1*0402) in the Japanese. Here, we will describe a typical case of a Laotian patient with VKH disease and the results of HLA typing: the patient showed a conservation of one of the major haplotypes observed in Japanese VKH patients, DRB1*0405-DQB1*0402.
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