Vogtkoyanagiharada syndrome vkh syndrome is an autoimmune disease in humans where ones own defense against infection, the tcells, attack the melaninforming cells melanocytes in the body. Vogtkoyanagiharada vkh prognosis depends on early recognition and treatment. Vogtkoyanagiharada vkh disease is a multisystem autoimmune inflammatory disorder with ocular, auditory, skin and neurologic involvement. Vogtkoyanagiharada syndrome is a rare disease, which probaly has a cellmediated autoimmune pathogenesis, marked by ocular anterior andor posterior uveitis, dermatological poliosis, canities, vitiligo and neurological meningoencephalitis disorders of variable severity in variable combinations. Vogt koyanagi harada disease vkh is a multisystem disease of presumed autoimmune cause that affects pigmented tissues, which have melanin. In 1906, alfred vogt in switzerland first described a patient with premature whitening of eyelashes of sudden onset and. The vogt koyanagi harada syndrome vkh is a bilateral, diffuse granulomatous uveitis associated with poliosis, vitiligo, alopecia, and central nervous system and auditory signs. Vogtkoyanagiharada disease is a rare disorder of unknown origin that affects many body systems, including as the eyes, ears, skin, and the covering of the brain and spinal cord the meninges. Vkh may variably also involve the inner ear, with effects on hearing, the skin and the meninges of the central nervous system. There may also be neurological signs such as severe headache, vertigo, nausea, and drowsiness. The vogt koyanagi harada syndrome vkhs is rare in the netherlands. Review article gender differences in vogtkoyanagiharada disease and sympathetic ophthalmia yujuanwang 1,2 andchichaochan 1 immunopathology section, laboratory of immunology, national eye institute, national institutes of health. Vogtkoyanagiharadas syndrome is a rare disease that affects tissues containing melanocytes, such as the eyes, central nervous system, inner ear and skin.
If an exudative choroiditis, causing retinal detachment, is present the condition is called. Citescore values are based on citation counts in a given year e. The vogt koyanagi harada vkh disease, formerly known as uveomeningitic syndrome, is a bilateral diffuse granulomatous panuveitis associated with exudative retinal detachment, which can be accompanied by central nervous system involvement, dermatological and auditory alterations. The vogtkoyanagiharada vkh disease, formerly known as uveomeningitic syndrome, is a bilateral diffuse granulomatous panuveitis associated with exudative retinal detachment, which can be accompanied by central nervous system involvement, dermatological and auditory alterations. Melanocytes are specialized cells that produce a pigment called melanin. Vogt koyanagi harada s syndrome is a rare disease that affects tissues containing melanocytes, such as the eyes, central nervous system, inner ear and skin. Vogtkoyanagiharada disease vkhd is a rare granulomatous inflammatory disease that affects pigmented structures, such as eye, inner ear. Pdf vogtkoyanagiharadas syndrome and its multisystem.
Vkh syndrome is named for ophthalmologists alfred vogt from switzerland and yoshizo. This disease is mainly a th1 lymphocyte mediated aggression to melanocytes after a viral trigger in the presence of hladrb10405 allele. An ebook reader can be a software application for use on a computer such as microsofts free reader application, or a booksized computer this is used solely as a reading device such as nuvomedias rocket ebook. Aug 17, 2018 vogtkoyanagiharada vkh disease is a multisystemic disorder characterized by granulomatous panuveitis with exudative retinal detachments that is often associated with neurologic and cutaneous manifestations. Several reports havea ppeared in veterinary literature in canada, in the united states and in united kingdom affecting different breeds. Aug 17, 2018 vogtkoyanagiharada vkh disease is a multisystem autoimmune inflammatory disorder with ocular, auditory, skin and neurologic involvement. Vogtkoyanagiharadas syndrome and its multisystem involvement.
All structured data from the file and property namespaces is available under the creative commons cc0 license. Vogtkoyanagiharada disease genetic and rare diseases nih. Apr 06, 2020 people with chs have light skin and silvery hair albinism and frequently complain of solar sensitivity and photophobia. Wagners syndrome an autosomal dominant inherited disease caused by mutation in the gene encoding chondroitin sulfate proteoglycan cspg2, which is a proteoglycan present in the vitreous humour. Some ethnic groups have a higher probability of developing the disease, including asians, indians and latin. The most significant manifestation is bilateral, diffuse uveitis, which affects the eyes. Vogt koyanagi harada s disease is a rare syndrome that affects tissues with melanocytes like eyes, central nervous system, skin and inner ear.
Vogtkoyanagiharadas disease is a rare syndrome that affects tissues with melanocytes like eyes, central. Mar 24, 2016 vogt koyanagi harada disease vkhd is a rare granulomatous inflammatory disease that affects pigmented structures, such as eye, inner ear, meninges, skin and hair. Melanin is what gives color, or pigment, to our hair, skin and parts of our eyes. The most noticeable symptom is a rapid loss of vision. Pdf a u g u s t 3 1, 2 0 1 0 v o l u m e 9 n u m b e r 1 6 contemporary. Its name derives from the authors who first described the disease. Vogtkoyanagiharada syndrome an overview sciencedirect. Dear iois members and esteemed congress delegates, i am delighted to welcome you all to the 14th meeting of the international ocular inflammation society and 4th international assembly of ocular inflammation societies, which will be held in lausanne, switzerland, a humansized charming european city.
Elejalde syndrome is an extremely rare autosomal recessive syndrome only around 10 cases known consisting of moderate pigment dilution, profound primary neurologic defects, no immune defects, and hair with metallic silvery sheen it is associated with myo5a see also. The conspicuous feature of the vogtkoyanagi syndrome is the severe, prolonged bilateral uveitis. It affects primarily asians, indians and latin americans and also women. Vogt koyanagi harada disease is a rare disorder of unknown origin that affects many body systems, including as the eyes, ears, skin, and the covering of the brain and spinal cord the meninges. The syndrome is characterised by a combination of panuveitis, pleocytosis in cerebral spinal fluid with or without neurological signs. Factors from saliva and oral bacteria, chemotactic for polymorphonuclear leukocytes. Vogtkoyanagiharada disease vkh is a multisystem disease of presumed autoimmune cause that affects pigmented tissues, which have melanin. Abstract we report a 37yearold woman with uveitic phase of vogt koyanagi harada disease and tonic pupil, the tonic pupil persisted after other clinical features of this syndrome had disappeared. Vogtkoyanagiharada disease vkh disease is a disease that affects several parts of the body, including the eyes, ears, nervous system, and skin. Vogtkoyanagiharada disease vkh is a multisystem disease of presumed autoimmune. Review article gender differences in vogtkoyanagiharada.
Elejalde syndrome is an extremely rare autosomal recessive syndrome only around 10 cases known consisting of moderate pigment dilution, profound primary neurologic defects, no immune defects, and hair with metallic silvery sheen. Susacs syndrome is a rare microangiopathy resulting in encephalopathy, retinopathy, and hearing loss, and is assumed to have an autoimmune basis. Vogt koyanagi harada syndrome is a rare disease, which probaly has a cellmediated autoimmune pathogenesis, marked by ocular anterior andor posterior uveitis, dermatological poliosis, canities, vitiligo and neurological meningoencephalitis disorders of variable severity in variable combinations. It is characterized by diffuse granulomatous inflammation involving various organs including eye. People with chs have light skin and silvery hair albinism and frequently complain of solar sensitivity and photophobia. Files are available under licenses specified on their description page. Vogtkoyanagiharada disease genetic and rare diseases.
Vogt koyanagi harada syndrome vkh is a disease distinguished by a triad of uveitis, dermatologic findings, and cns involvement. The patient was started on oral and topical steroids. Vkh disease occurs more commonly in patients with a genetic predisposition to the disease, including those from asian, middle eastern. Vogtkoyanagiharada syndrome nederlands tijdschrift. Vogtkoyanagiharada disease vkhd, initially described as an uveomeningoencephalitic syndrome, is a systemic granulomatous autoimmune disease that targets melanocyterich tissues, such as the eye, inner ear, meninges, skin and hair. The term vogtkoyanagiharada disease was coined by bruno and. The vogtkoyanagiharada syndrome vkhs is rare in the netherlands. The conspicuous feature of the vogt koyanagi syndrome is the severe, prolonged bilateral uveitis. Review article gender differences in vogt koyanagi harada disease and sympathetic ophthalmia yujuanwang 1,2 andchichaochan 1 immunopathology section, laboratory of immunology, national eye institute, national institutes of health. Vogt koyanagi harada syndrome is a multisystem disorder characterized by granulomatous panuveitis with exudative retinal detachments that is often associated with neurologic and cutaneous manifestations. Introduction the vogtkoyanagiharada vkh syndrome is a bilateral, diffuse, granulomatous uveitis associated with vitiligo depigmentation of the skin, poliosis hair whitening, alopecia, and signs of meningeal irritation with or without auditory disturbances which was first described in humans by vogt 1906, harada 1926, and koyanagi 1929. Vogtkoyanagiharada syndrome is a rare condition characterized by bilateral uveitis with cutaneous and neurologic features cerebrospinal fluid pleocytosis and auditory abnormalities. Despite the initial treatment, the patient progressed to.
A similar syndrome was firstreported in japan in akita dogs. Melanin is the substance that gives skin, hair, and eyes their color. Vogtkoyanagiharada disease nord national organization for. Some ethnic groups have a higher probability of developing the disease, including asians, indians and latin americans and females are affected more often. Pdf on aug 1, 2014, emmett t cunningham and others published. Vogtkoyanagiharada disease johns hopkins university. Uveitis and dermal depigmentation vogtkoyanagiharada1. Rituximab in refractory vogtkoyanagiharada disease springerlink. Syndrome definition of syndrome by medical dictionary.
The condition is characterized by an empty vitreous cavity or dense membranes within the vitreous. Vogtkoyanagiharada syndrome radiology reference article. Vogt koyanagi harada is a systemic inflammatory disease affecting the eyes, the central nervous system and the skin, and it is caused by an autoimmune reaction against antigens associated with melanocytes. Vogtkoyanagiharada syndrome nederlands tijdschrift voor. Increased peripheral blood cd4plus cd8plus tcell ratios have been seen in vkh, suggesting an immunemediated disease. Vogtkoyanagiharada syndrome with more emphasis on the. Vogtkoyanagiharada vkh disease is a multisystemic disorder characterized by granulomatous panuveitis with exudative retinal detachments that is often associated with neurologic and cutaneous manifestations. The signs and symptoms of vkh disease are caused by chronic inflammation of melanocytes. Vogtkoyanagiharada vkh disease clinical presentation. The clinical pattern in the case reported here showed severe neurological involvement. The symptoms of the vogtkoyanagi syndrome overlap those of harada diseasesyndrome, and they may at times be indistinguishable from haradas disease. Vkh disease occurs more commonly in patients with a genetic predisposition to the disease, including those from asian, middle eastern, hispanic, and native american populations. A collection of disease information resources and questions answered by our genetic and rare diseases information specialists for vogtkoyanagiharada. Vogtkoyanagiharada syndrome vkh syndrome is an uncommon multisystem disease of presumed autoimmune etiology that is characterized by chronic, bilateral, diffuse, granulomatous uveitis with accompanying dermatologic, neurologic, and auditory involvement.
The syndrome is characterised by a combination of panuveitis, pleocytosis in cerebral spinal fluid with or without neurological signs, alopecia and depigmentation of body hair. Oct 26, 2017 vogt koyanagi harada disease vkh disease is a disease that affects several parts of the body, including the eyes, ears, nervous system, and skin. However, in asia it is one of the most common causes of panuveitis. Vogtkoyanagiharada vkh disease is a multisystemic disorder characterized by granulomatous panuveitis with exudative retinal. The vogt koyanagi harada syndrome have been studied in human beings. Uveitis and dermal depigmentation vogtkoyanagiharada1 ike.
The vogtkoyanagiharada syndrome vkh is a bilateral, diffuse granulomatous uveitis associated with poliosis, vitiligo, alopecia, and central nervous system and auditory signs. Vogt koyanagi harada syndrome sharmeen akram and khabir ahmad abstract vogt koyanagi harada vkh syndrome is a rare multisystem disease of melanocyte containing organs. The symptoms of the vogt koyanagi syndrome overlap those of harada diseasesyndrome, and they may at times be indistinguishable from harada s disease. The vogtkoyanagiharada syndrome have been studied in human beings. Vogtkoyanagiharada vkh disease is defined as a bilateral granulomatous panuveitis with or without extraocular manifestations affecting. Vogtkoyanagiharada disease nord national organization.
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