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.     

Vogt-Koyanagi-Harada Disease

Vogt-Koyanagi-Harada disease (VKH) is a multisystem autoimmune disorder principally affecting pigmented tissues in the ocular, auditory, integumentary and central nervous systems. Patients are typically 20 to 50 years old and have no history of either surgical or accidental ocular trauma. Pigmented races are more commonly affected. Depending on revised diagnostic criteria, the disease is classified as complete, incomplete or probable based on the presence of extraocular findings (neurological, auditory and integumentary). The clinical course of VKH is divided into four phases: prodromal (mimics a viral infection), uveitic (bilateral diffuse uveitis with papillitis and exudative retinal detachment), convalescent (tissue depigmentation), and chronic recurrent (recurrent uveitis and ocular complications). The pathogenesis of VKH is thought to be related to an aberrant T cell-mediated immune response directed against self-antigens found on melanocytes. VKH has been linked to human leukocyte antigen DR4 (HLA-DR4) and HLA-Dw53, with strongest associated risk for HLA-DRB1*0405 haplotype. The diagnosis of VKH is clinical, and differential includes sympathetic ophthalmia, sarcoidosis, primary intraocular B-cell lymphoma, posterior scleritis, and uveal effusion syndrome. Treatment is typically initiated with high-dose oral corticosteroids, but other immunomondulatory agents (most oftentimes cyclosporine) may be needed for non-responsive patients or when corticosteroid side-effects are not tolerated. Visual prognosis is generally good with prompt diagnosis and aggressive immunomodulatory treatment.     

Vogt-Koyanagi-Harada disease

Vogt-Koyanagi-Harada disease (VKH) is a multisystem autoimmune disorder principally affecting pigmented tissues in the ocular, auditory, integumentary and central nervous systems. Patients are typically 20 to 50 years old and have no history of either surgical or accidental ocular trauma. Pigmented races are more commonly affected. Depending on revised diagnostic criteria, the disease is classified as complete, incomplete or probable based on the presence of extraocular findings (neurological, auditory and integumentary). The clinical course of VKH is divided into four phases: prodromal (mimics a viral infection), uveitic (bilateral diffuse uveitis with papillitis and exudative retinal detachment), convalescent (tissue depigmentation), and chronic recurrent (recurrent uveitis and ocular complications). The pathogenesis of VKH is thought to be related to an aberrant T cell-mediated immune response directed against self-antigens found on melanocytes. VKH has been linked to human leukocyte antigen DR4 (HLA-DR4) and HLA-Dw53, with strongest associated risk for HLA-DRB1*0405 haplotype. The diagnosis of VKH is clinical, and differential includes sympathetic ophthalmia, sarcoidosis, primary intraocular B-cell lymphoma, posterior scleritis, and uveal effusion syndrome. Treatment is typically initiated with high-dose oral corticosteroids, but other immunomondulatory agents (most oftentimes cyclosporine) may be needed for non-responsive patients or when corticosteroid side-effects are not tolerated. Visual prognosis is generally good with prompt diagnosis and aggressive immunomodulatory treatment.     

Vogt-Koyanagi-Harada syndrome with intracranial meningioma: an as yet unreported association

CASE REPORT: We document a previously unreported association of Vogt-Koyanagi-Harada (VKH) syndrome with intracranial meningioma. A female patient with diminished vision, exudative retinal detachment, and headache was diagnosed with VKH syndrome, more precisely a Harada form of disease with intracranial meningioma, on the basis of exudative retinal detachment, typical fundus fluorescein findings, and magnetic resonance imaging. With intravenous steroid therapy, visual acuity improved and the detachment settled within a week. At 3 months, the detachment recurred but improved after retreatment. At 1 year, the tumour was unchanged in size. COMMENTS: VKH syndrome may be associated with intracranial meningioma that may affect the patient's overall morbidity or mortality.     

Initial-onset acute uveitis associated with Vogt-Koyanagi-Harada disease presenting with unilateral exudative retinal detachment despite bilateral choroidal involvement

International Ophthalmology - To investigate the frequency of initial-onset acute uveitis associated with Vogt-Koyanagi-Harada (VKH) disease presenting with unilateral exudative retinal detachment....     

Sympathetic Ophthalmia

Sympathetic ophthalmia is a rare, bilateral granulomatous uveitis that occurs after either surgical or accidental trauma to one eye. The ocular inflammation in the fellow eye becomes apparent usually within 3 months after injury. Clinical presentation is an insidious or acute anterior uveitis with mutton-fat keratic precipitates. The posterior segment manifests moderate to severe vitritis, usually accompanied by multiple yellowish-white choroidal lesions. Evidence suggests that sympathetic ophthalmia represents an autoimmune inflammatory response against choroidal melanocytes mediated by T cells. Diagnosis is based on clinical findings and a history of previous ocular trauma or surgery. Other causes of granulomatous uveitis, such as Vogt-Koyanagi-Harada disease, sarcoidosis, tuberculosis, and syphilis should be considered. Treatment of sympathetic ophthalmia consists of systemic anti-inflammatory agents with high dose oral corticosteroid as the drug of choice. However, if the inflammation cannot be controlled, cyclosporine is then used. Other immunosuppressive agents, such as chlorambucil, cyclophosphamide or azathioprine, may be necessary for the control of inflammation. The role of enucleation after the diagnosis of sympathetic ophthalmia remains controversial. Visual prognosis is reasonably good with prompt wound repair and appropriate immunomodulatory therapy.     

Systemic non-Hodgkin's lymphoma masquerading as Vogt-Koyanagi-Harada disease in an HIV-positive patient

A 29-year-old man presented with decreased visual acuity in both eyes secondary to exudative retinal detachment resembling Vogt-Koyanagi-Harada disease. Although fluorescein angiographic pictures supported the clinical findings, there was no choroidal thickening evident with ultrasonography. In 3 days he developed increased disc oedema with peripapillary haemorrhages in both eyes. Further evaluation revealed HIV-positive status and a systemic non-Hodgkin's lymphoma. The patient responded favourably to the treatment for systemic non-Hodgkin's lymphoma confirming our diagnosis of intraocular metastasis. In bilateral exudative detachment, an absence of choroidal thickening on ultrasonography and the presence of peripapillary haemorrhages should prompt a systemic evaluation for causes other than Vogt-Koyanagi-Harada disease, especially in HIV-positive patients.     

Sympathetic ophthalmia

Sympathetic ophthalmia is a rare, bilateral granulomatous uveitis that occurs after either surgical or accidental trauma to one eye. The ocular inflammation in the fellow eye becomes apparent usually within 3 months after injury. Clinical presentation is an insidious or acute anterior uveitis with mutton-fat keratic precipitates. The posterior segment manifests moderate to severe vitritis, usually accompanied by multiple yellowish-white choroidal lesions. Evidence suggests that sympathetic ophthalmia represents an autoimmune inflammatory response against choroidal melanocytes mediated by T cells. Diagnosis is based on clinical findings and a history of previous ocular trauma or surgery. Other causes of granulomatous uveitis, such as Vogt-Koyanagi-Harada disease, sarcoidosis, tuberculosis, and syphilis should be considered. Treatment of sympathetic ophthalmia consists of systemic anti-inflammatory agents with high dose oral corticosteroid as the drug of choice. However, if the inflammation cannot be controlled, cyclosporine is then used. Other immunosuppressive agents, such as chlorambucil, cyclophosphamide or azathioprine, may be necessary for the control of inflammation. The role of enucleation after the diagnosis of sympathetic ophthalmia remains controversial. Visual prognosis is reasonably good with prompt wound repair and appropriate immunomodulatory therapy.     

Management of ocular complications of Vogt-Koyanagi-Harada syndrome

Background Vogt-Koyanagi-Harada syndrome (VKH) is a multisystem disorder, characterized by the T-cell-mediated autoimmune process directed against melanocytic antigens in the ocular, nervous, auditory and integumentary systems. The ocular hallmarks of the disease involve severe bilateral panuveitis associated with exudative retinal detachment. Case report We report a pediatric case of probable VKH Syndrome with isolated ocular findings, in which bilateral vitritis, papillitis and serous retinal detachments involving the macula with intra-retinal edema and choroideal thickening were detected. Intervention Subtenon triamcinolone acetonide injection was performed in addition to systemic corticosteroid and cyclosporine treatments. Evolution Prompt improvement was seen in the ocular manifestations of VKH syndrome, which persisted for at least 4 months. Subtenon corticosteroid injection, together with systemic corticosteroid and cytotoxic treatment, prevented the ocular complications of invasive intraocular treatment modalities, and at the same time reduced the systemic corticosteroid dosage. Conclusion We recommend use of ocular invasive treatment modalities in Vogt-Koyanagi-Harada Syndrome only if the disease progression cannot be controlled. If systemic medications fail to stop the progression of the disease activity, subtenon injections may be considered before intraocular treatment modalities. The authors have full control of all primary data and they will allow International Ophthalmology to review their data upon request.     

Sympathische Ophthalmie

Sympathetic ophthalmia is a rare, bilateral granulomatous uveitis that occurs after either surgical or accidental trauma to one eye. The etiology is still not completely clear, but evidence suggests that sympathetic ophthalmia represents an autoimmune inflammatory response against choroidal melanocytes mediated by T cells. Key features are vision impairment and symptoms associated with inflammation. The diagnosis is based on a history of previous ocular trauma or surgery and clinical findings. Differential diagnoses include other causes of granulomatous uveitis, such as Vogt-Koyanagi-Harada disease, sarcoidosis, tuberculosis, and syphilis. Depending on the suspected etiology, treatment of sympathetic ophthalmia consists of systemic anti-inflammatory agents, including corticosteroids and immunomodulating drugs. The role of enucleation after the diagnosis of sympathetic ophthalmia remains controversial. Visual prognosis is reasonably good with prompt appropriate wound repair and immunomodulatory therapy. As the occurrence of sympathetic ophthalmia is probably more frequent following vitreoretinal surgery, more attention has to be paid to this potentially bilateral blinding disorder.     

Retinal pigment epithelial tear and extensive exudative retinal detachment following blunt trauma

Background: A peripheral retinal pigment epithelial tear and an extensive exudative retinal detachment caused by choroidal leakage from the denuded Bruch’s membrane are extremely rare. A peripheral retinal pigment epithelial tear has not been reported in an eye with retinochoroidal folds after blunt ocular trauma. Methods: Case report. Results: The course of a large nasal peripheral retinal pigment epithelial tear that occurred after blunt ocular trauma in a patient with retinochoroidal folds was followed. The inferior retinal detachment caused by leakage from the denuded Bruch’s membrane following the development of the tear gradually worsened. Initial treatment with cryotherapy was ineffective, but the retinal detachment eventually resolved after the patient underwent sclerectomy and sclerostomy. Conclusion: A large peripheral retinal pigment epithelial tear can occur in patients with retinochoroidal folds following blunt ocular trauma, and extensive retinal detachment can be induced. Sclerectomy and sclerostomy can be beneficial in patients with an extensive exudative retinal detachment caused by choroidal leakage from the denuded Bruch’s membrane. Received: 12 October 1999 Revised: 6 January 2000 Accepted: 9 March 2000     

Acute Lymphoblastic Leukemia Manifesting as Acute Vogt-Koyanagi-Harada Disease

We describe a case of bilateral exudative retinal detachment associated with prodromal symptoms simulating the presentation of acute Vogt-Koyanagi-Harada disease that was eventually diagnosed as acute lymphoblastic leukemia. A 42-year-old man presented with sudden visual loss in both eyes for two weeks. He complained of intermittent headache, neck stiffness and tinnitus for a month. His best-corrected visual acuities were 20/200 in both eyes. Fluorescein angiography, optical coherence topography and indocyanine green angiography featured bilateral serous retinal detachments. A clinical diagnosis of incomplete type Vogt-Koyanagi-Harada disease was considered. However, complete blood cell count showed a marked increase in the number of white blood cells and bone marrow examination revealed precursor B cell lymphoblastic leukemia. The patient started on induction chemotherapy. A week later, his best-corrected visual acuities were 20/25 and the serous retinal detachments were nearly absorbed in both eyes. Bilateral exudative retinal detachment associated with neurologic and auditory abnormalities may be a presenting sign of acute lymphoblastic leukemia. Clinicians should be aware of the possibility of leukemia in such patients.     

梅毒性葡萄膜炎3例

目的:评估梅毒性葡萄膜炎患者的临床表现和视力。方法:梅毒性葡萄膜炎患者3例在USM医院接受治疗。结果:患者3例被诊断为继发性梅毒性葡萄膜炎,这3例患者患病之前均不知道患有梅毒,但他们有明确的乱交史。并且每个月都伴有逐步的视力下降。其中两人伴有发热、眼痛、眼前悬浮物。视力从6/12到手动。所有患者均出现前葡萄膜炎,玻璃体炎和视神经炎。第一例患者出现了多灶性脉络膜视网膜炎,并伴有渗出性视网膜脱离。第二例患者出现渗出性视网膜脱离,而第三例患者仅出现了脉络膜视网膜炎。所有患者每周注射苄青霉素2.4MU,共4wk,其中2例患者口服多西霉素200mg2次/d,共3mo。治疗效果良好,其中2例患者有显著的视力上升,分别从6/120到6/21和6/12到6/6。其中较严重的1例患者出现逆转录酶为阳性。结论:眼梅毒作为非肉芽肿性的炎症与渗出性视网膜脱离有关。治疗后虽然视力恢复比较缓慢,但普遍有良好的效果。     

A report of two cases suggesting positive influence of pregnancy on uveitis activity

BACKGROUND: Little is known about the influence of pregnancy on uveitis activity. We report two cases suggesting a favorable influence of pregnancy on the clinical course of uveitis. CASE: A 30-year-old woman who was three months pregnant was suspected Vogt-Koyanagi-Harada disease based on the systemic symptoms and ocular findings of iritis and multi-focal serous retinal detachment. She was positive to human leukocyte antigen (HLA)-DR 4. She was treated only with topical corticosteroids. One month later, the retinal detachment disappeared. Six month later, a healthy baby was born. The fundus of both eyes took on a sunset glow appearance and there has been no recurrence of uveitis. The other case was a 23-year-old woman with Beh?et's disease who had several episodes of uveitis in a year even on tacrolimus. Because of pregnancy, all systemic drugs including tacrolimus were discontinued since then. Interestingly, the frequency of uveitis was remarkably decreased during the pregnancy. A normal healthy baby was born. The uveitis has almost completely disappeared since parturition until now. CONCLUSION: It is considered that the increase of intrinsic hormone, especially corticosteroid, and some other factors with pregnancy may give the suppressive influence on uveitis in our cases.     

Tuberculin skin-test-induced uveitis in the absence of tuberculosis

A 16-year-old Caucasian girl who showed no evidence of tuberculosis or other systemic disease developed acute panuveitis progressing to bilateral serous retinal detachments following purified protein derivative (PPD) skin testing on two separate occasions separated by an interval of 8 years. Both episodes responded rapidly to steroid therapy. Uveal hypersensitivity to PPD skin testing has been described in the presence of ocular or systemic tuberculosis, Eales' disease, and the Vogt-Koyanagi-Harada syndrome (VKH). Our case, however, suggests that profound uveitis may be precipitated by routine PPD skin testing alone. Offprint requests to: C.F. Burgoyne     

Conjunctival nodules associated with Vogt-Koyanagi-Harada disease

Background To report on conjunctival nodules as an unusual manifestation of Vogt-Koyanagi-Harada disease. Methods A 24-year-old woman presented with a two-month history of bilateral conjunctival injection and gradually decreased vision. Ophthalmological examinations revealed bilateral granulomatous uveitis and bulbar conjunctival nodules, and a biopsy of the conjunctival nodules was performed. Results The biopsy specimens showed noncaseating granulomas. The major components of the infiltrating lymphocytes were CD8-positive T cells. Topical corticosteroid therapy reduced the anterior segment inflammation, and the conjunctival nodules disappeared within a week. Afterwards, the patient demonstrated bilateral retinal detachments, sunset glow fundus and alopecia, and, therefore, was diagnosed to have Vogt-Koyanagi-Harada disease four months after the first symptoms. Conclusions Conjunctival nodules may represent the primary manifestation of Vogt-Koyanagi-Harada disease. Financial disclosure: none.     

Exudative retinal detachment and retinitis associated with acquired syphilitic uveitis

PURPOSE: To describe three cases of exudative retinal detachment and focal retinitis associated with acquired syphilitic uveitis. METHODS: Three patients who were referred for evaluation of uveitis were examined. Slit-lamp examination, ophthalmoscopy, B-scan ultrasonography, fundus photography, and fluorescein angiography were performed before and after therapy. RESULTS: Each patient had uveitis with exudative retinal detachment, periphlebitis, and focal retinitis. Laboratory testing (fluorescent treponemal antibody absorption) revealed positive serology for active syphilis in all cases. Human immunodeficiency virus antibody testing was negative in all patients. Retinal detachment resolved in all cases after treatment with intravenous penicillin. Despite resolution of subretinal fluid, visual acuity remained poor in eyes in which the macula was detached. CONCLUSION: Syphilis is a cause of exudative retinal detachment. Antibiotic therapy can lead to retinal reattachment. Early recognition and treatment may prevent severe vision loss.     

Syphilitic uveitis: report of 3 cases

AIM: To evaluate the clinical manifestations and visual outcome of syphilitic uveitis patients. METHODS: Case series of three patients with syphilitic uveitis who were managed in Hospital USM. RESULTS: Three patients were diagnosed to have uveitis secondary to Syphilis. All three patients were not known to have syphilis prior to presentation but have positive history of sexual promiscuity. All patients presented with progressive blurring of vision for average of one-month duration. Two of them have association with fever, ocular pain and floaters. Visual acuity at presentation ranges from 6/12 to hand movement. Mild anterior uveitis (non-granulomatous), vitritis and papillitis were presence in all the patients. First patient has multifocal chorioretinitis with exudative retinal detachment. The second patient presented with exudative retinal detachment while the third patient has chorioretinitis only. All the patients were treated with intramuscular benzyl-penicillin 2.4 MU weekly for 4 weeks and two of them received oral doxycycline 200mg twice daily for 3 months. The uveitis responded well to the treatment and two of them showed dramatic visual improvement from 6/120 to 6/21 and 6/12 to 6/6. The one with worse outcome was confirmed to have positive retroviral. CONCLUSION: Ocular syphilis presented here as non- granulomatous inflammation associated with exudative retinal detachment. Final visual outcome is generally good despite slow improvement after treatment.     

Simultaneous bilateral retinal detachment following coronary artery bypass graft: case report

PURPOSE: To present an unusual case of simultaneous bilateral retinal detachment (RD) following a coronary artery bypass graft in a patient with acute myocardial infarction (AMI). METHODS: A 78-year-old man was first seen for bilateral sudden visual loss after surgical treatment of AMI. The patient underwent ultrasound biomicroscopy (UBM) and ocular B-scan echographic examination. RESULTS: The ocular assessment showed a bilateral seclusion of the pupil with bombe of the iris, an anterior chamber without cells or flare, and hypotonia. The evaluation of the visual acuity revealed no light perception in the right eye (RE) and uncertain light perception in the left eye (LE). The UBM analysis of the anterior segment confirmed the presence of bilateral pupillary block due to the seclusion of the pupil and a peripheral serous choroidal detachment involving the RE. The echographic B-scan analysis of the posterior segment showed a bilateral closed funnel-shaped RD and confirmed the presence of the peripheral flat serous choroidal detachment in RE. CONCLUSIONS: The cause for simultaneous bilateral RD remained unclear. It may have been a consequence of a persistent choroidal detachment with multiple swelling and 'kissing' of retinal surface. The increased venous pressure caused by congestive heart failure due to AMI could have caused a bilateral uveal effusion. Alternatively, the absence of retinal tears, the presence of a closed funnel-shaped morphology, and seclusion of the pupils allowed us to suspect an exudative pathogenetic mechanism due to a previous unrecognized ocular inflammatory state.     

梅毒性葡萄膜炎3例

目的：评估梅毒性葡萄膜炎患者的临床表现和视力. 方法：梅毒性葡萄膜炎患者3例在USM医院接受治疗. 结果：患者3例被诊断为继发性梅毒性葡萄膜炎,这3例患者患病之前均不知道患有梅毒,但他们有明确的乱交史.并且每个月都伴有逐步的视力下降.其中两人伴有发热、跟痛、眼前悬浮物.视力从6/12到手动.所有患者均出现前葡萄膜炎,玻璃体炎和视神经炎.第一例患者出现了多灶性脉络膜视网膜炎,并伴有渗出性视网膜脱离.第二例患者出现渗出性视网膜脱离,而第三例患者仅出现了脉络膜视网膜炎.所有患者每周注射苄青霉素2.4MU,共4wk,其中2例患者口服多西霉素200mg 2次/d,共3mo.治疗效果良好,其中2例患者有显著的视力上升,分别从6/120到6/21和6/12到6/6.其中较严重的1例患者出现逆转录酶为阳性. 结论：眼梅毒作为非肉芽肿性的炎症与渗出性视网膜脱离有关.治疗后虽然视力恢复比较缓慢,但普遍有良好的效果.