1/113. Vogt-Koyanagi-Harada disease: extensive vitiligo with prodromal generalized erythroderma. The occurrence of vitiligo with prodromal erythema in Vogt-Koyanagi-Harada (VKH) disease is rarely observed. We describe a 68-year-old Chinese woman who developed extensive vitiligo 6 months after the onset of a generalized erythroderma and 12 months after the onset of acute bilateral uveitis. She also had a 4-year history of psoriatic plaques on the trunk which resolved completely without recurrence 15 months after the onset of erythroderma. The extensive cutaneous inflammation prior to the onset of vitiligo in our patient with VKH disease has not been reported before and strongly suggests an immunologic etiology for VKH disease. ( info) |
2/113. 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. ( info) |
3/113. headache and bilateral visual loss in a young hypothyroid Indian man. We describe the exceptional association of Vogt-Koyanagi-Harada syndrome (VKHS) and hypothyroidism in a 29-year-old man of Indian heritage. VKHS is a rare uveomeningoencephalitic syndrome with probably autoimmune pathogenesis. Nontraumatic uveitis, aseptic meningoencephalitis, vitiligo, alopecia and poliosis are the leading clinical features of VKHS. The reported patient presented with bilateral visual loss and progressive frontal headache. VKHS was diagnosed due to characteristic ophthalmological findings and the diagnosis of aseptic meningitis. Due to the autoimmune pathogenesis, VKHS may be rarely associated with other autoimmune disorders. association of VKHS with autoimmune thyroid disease has been described in the literature in three patients. In the reported case hypothyroidism due to chronic autommune thyroiditis was diagnosed in association with VKHS. Routinely determination of thyroid function in patients with VKHS is recommended. ( info) |
4/113. 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. ( info) |
5/113. 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. ( info) |
6/113. 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. ( info) |
7/113. Intrafamilial occurrence of tubulointerstitial nephritis with uveitis and Vogt-Koyanagi-Harada syndrome. We describe a child with acute tubular dysfunction, reversible renal failure, and uveitis, whose father has Vogt-Koyanagi-Harada syndrome. This is the first reported familial association of these 2 syndromes with distinctively overlapping clinical characteristics. A pathogenic association is proposed. ( info) |
8/113. eye disease in a patient with rheumatoid arthritis. We report the case of a 40-year-old woman with diffuse uveitis, sensorineural hearing loss and cerebrospinal fluid pleocytosis as features of Vogt-Koyanagi-Harada syndrome who developed symmetric polyarthritis and stiffness of small and large joints, in addition to rheumatoid arthritis. Although their target tissues are distinct, both diseases have a possible autoimmune origin strongly associated with HLA-DRB4. ( info) |
| BACKGROUND: To describe clinical findings of subretinal fibrosis and choroidal neovascularization in patients with Vogt-Koyanagi-Harada (VKH) syndrome. methods: We retrospectively reviewed 75 medical records of patients with VKH seen at the National eye Institute, Bethesda, maryland between 1978 and 1996. Recorded data included age, gender, race, duration of disease, extraocular manifestations, best-corrected visual acuity, slit-lamp biomicroscopy, retinal examination, retinal photographs and fluorescein angiograms. We sought features that correlated with the visual outcome. RESULTS: Thirty of 75 (40%) patients developed subretinal fibrosis. Eleven patients (14.7%) had choroidal neovascularization. Presence of subretinal fibrosis was associated with a longer duration of the disease (42.6 vs 19.1 months, P = 0.07). patients with subretinal fibrosis had worse visual acuity than those without subretinal fibrosis (26.2 vs 57.3 ETDRS letters read, P < 0.001) after adjusting for duration of disease (P = 0.021), degree of vitreous haze (P = 0.074), and use of immunosuppressive therapy (P = 0.008). CONCLUSIONS: Presence of subretinal fibrosis in patients with VKH is associated with a poor visual prognosis. The diagnosis of choroidal neovascularization and subretinal fibrosis presents a challenge in the management of this disease. ( info) |
10/113. Vogt-Koyanagi-Harada syndrome in a pregnant patient treated with high-dose systemic corticosteroids. PURPOSE: Vogt-Koyanagi-Harada (VKH) syndrome is usually treated with high-dose systemic corticosteroids. However, in a pregnant patient with VKH syndrome, the effects of high-dose corticosteroids on the fetus are controversial. We discuss treating VKH syndrome during pregnancy, systemic corticosteroids, and abnormalities in the delivered infant. methods: Case report and literature review. RESULTS: A 26-year-old Japanese woman in the 16th week of gestation was diagnosed with VKH syndrome and treated with high-dose systemic prednisolone after the 18th week of gestation. Although the patient's ocular findings gradually improved, a low-birth-weight infant was delivered with epibulbar dermoid, lipodermoids, and preauricular appendages. Although low birth weight might result from systemic prednisolone administration, the anomalies of the infant may have been inherited rather than caused by VKH syndrome and high-dose systemic corticosteroid administration. CONCLUSION: In this case and previously reported cases, VKH syndrome and systemic corticosteroids administered during pregnancy may not precipitate abortion and congenital malformation. ( info) |