In both, the disease appeared during the second half of pregnancy with complete remission. Med any combination of signs and symptoms that are indicative of a particular disease or disorder syndrome, mr. Association between vogtkoyanagiharada syndrome and hladr1 and dr4 in hispanic patients living in southern california. Find out information about vogtkoyanagiharada syndrome.
A case of probable vogtkoyanagiharada disease in a 3yearold. Research suggests it may be due to an autoimmune reaction in which the body attacks its own healthy cells that contain the pigment melanin. Introduction vogtkoyanagiharada vkh disease is a multisystemic disorder characterized by granulomatous panuveitis with exudative retinal detachments that is often associated with neurologic and cutaneous manifestations. 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 vogtkoyanagiharada syndrome vkh is a bilateral, diffuse granuloma tous uveitis associated with poliosis, vitiligo, alopecia, and central nervous system and auditory signs. Recurrent anterior uveitis in patients with vogtkoyanagi. 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. Uveomeningoencephalitic syndrome vogtkoyanagiharada. The vogtkoyanagiharada vkh syndrome is a rare systemic disorder of uveitis, dysacousia, vitiligo, premature graying of the hair, eyebrows and eyelashes, and meningoencephalitis. A similar disorder with uveitis and depigmentation of the nose, lips, eyelids, footpads and anus occurs in dogs. Vogtkoyanagiharadas syndrome is a rare disease that affects tissues containing melanocytes, such as the eyes, central nervous system, inner ear and skin.
Vogtkoyanagiharada disease vkh is a wellestablished multiorgan disorder that affects pigmented structures, such as the eye, inner ear, meninges, and skin 1. Vogtkoyanagiharada vkh disease is a bilateral granulomatous choroiditis that invariably starts in the choroidal stroma and is therefore called a primary stromal. Differential diagnosis of vogtkoyanagiharada syndrome. Get a printable copy pdf file of the complete article 765k, or click on a page image below to browse page by page. This casecontrol study develops and evaluates a set of diagnostic criteria for vogtkoyanagiharada disease, an immunemediated disorder.
The classic course of vkh syndrome has three phases. Vogtkoyanagiharada syndrome vkhs is a rare multisystemic autoimmune disease that affects tissues containing melanin, including the eye, inner ear, meninges, and skin. Development and evaluation of diagnostic criteria for vogt. 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. It is an inflammatory condition of autoimmune nature in which cytotoxic t cell target melanocytes in genetically susceptible individuals. Two cases of the vogt koyanagi harada syndrome occurring during pregnancy are reported for the first time. Read rw, holland gn, rao na, tabbara kf, ohno s, criteria, patients may present with a complete, an in arellanesgarcia l, pivettipezzi p, tessler hh, usui m. Neurological involvement typical of vkh syndrome 8 th cranial nerve damage and sterile meningitis. Alezzandrini syndrome has been closely associated with vogtkoyanagiharada vkh syndrome, as they share similar cutaneous and ocular signs. The exact cause of vogt koyanagi harada vkh disease is not well understood, but research suggests it is an autoimmune disease in which the body attacks its own pigment cells melanocytes, possibly in response to an infection such as a virus. Vogtkoyanagiharada disease johns hopkins university. Vogtkoyanagiharada disease genetic and rare diseases.
Vogtkoyanagiharada vkh syndrome is a bilateral granulomatous panuveitiswith central nervous system, auditory, and integumentary manifestations. Vogtkoyanagiharada vkh disease is a bilateral granulomatous uveitis often associated with exudative retinal detachment and with extraocular manifestations, such as pleocytosis in the cerebrospinal fluid and, in some cases, vitiligo, poliosis, alopecia, and dysacusis. It is a bilateral granulomatous panuveitis associated with dermatologic and neurologic manifestations, including vitiligo, alopecia, poliosis, and meningeal signs. Vogtkoyanagiharada vkh syndrome is a rare multisystemic disease of presumed autoimmune etiology, affecting various melanocytecontaining organs. Vogtkoyanagiharada syndrome is a granulomatous autoimmune disease involving central nervous system, eyes, skin, and ears. Echographic features of the vogtkoyanagiharada syndrome. These manifestations are variable and race dependent. Indocyanine green angiography guided management of vogt. Vogtkoyanagiharada syndrome with more emphasis on the. Melanin is the substance that gives skin, hair, and eyes their color.
Vogtkoyanagiharada vkh disease clinical presentation. We report a case of a 10 year old girl who presented with. Stephen foster, md introduction vogtkoyanagiharada vkh disease, also known as uveomeningitic syndrome, is an idiopathic multisystem in. Vogtkoyanagiharada syndrome vkh is a systemic inflammatory disorder that predominantly affects individuals with darker skin pigmentation and shows a slight female predilection. Vogtkoyanagiharada syndrome current perspectives opth. Visual outcomes were good, with final visual acuity of better than 2030 in 29 66% of 44 eyes and of worse than 20400 in only three 7% of. The word symptoms of vogtkoyanagiharada syndrome is the more general meaning. We describe a 20yearold britishhonduran man with recent worsening headache and photophobia, vomiting and visual blurring. The phrase signs of vogtkoyanagiharada syndrome should, strictly speaking, refer only to those signs and symptoms of vogtkoyanagiharada syndrome that are not readily apparent to the patient.
The authors used a standard microlymphocyte cytotoxicity assay to type 9 patients with the vogt koyanagi harada syndrome for 25 hla a and b antigens, including bw22j. Although frequently unrecognised,vkh may affect children. Acute vogtkoyanagiharada vkh disease and acute central serous chorioretinopathy cscr are two common disorders with serous retinal detachment caused by dysfunction of choroid. The authors used a standard microlymphocyte cytotoxicity assay to type 9 patients with the vogt koyanagi harada syndrome. Vogtkoyanagiharada disease vkh is a multisystem disease of presumed autoimmune cause that affects pigmented tissues, which have melanin. Some authors distinguish between the vogtkoyanagi syndrome and the harada disease. Vogtkoyanagiharada disease is a bilateral, chronic, diffuse granulomatous panuveitis typically characterized by serous retinal detachment and frequently associated with neurological meningitis, auditory, and dermatological alterations. Full text full text is available as a scanned copy of the original print version.
Vogtkoyanagiharada disease nord national organization. Pdf vogtkoyanagiharada syndrome is a bilateral, chronic, diffuse granulomatous. Uveomeningoencephalitic syndrome vogtkoyanagi haranda vkh is an unusual syndrome with important neurological complications and has not, to my knowledge, been discussed in english in the neurological literature. It is characterized by diffuse granulomatous inflammation involving various organs including eye. The aetiology of the disease is still not known although recent findings point to an autoimmune reaction against. Vogtkoyanagiharada disease, a severe bilateral granulomatous intraocular inflammation associated with serous retinal detachments, disk edema, and vitritis, with eventual development of a sunset glow fundus, is an autoimmune inflammatory condition mediated by t cells that target melanocytes in individuals susceptible to the disease. Comparison of the optical coherence tomographic characters. Vogt koyanagi harada disease is a rare, multisystem, autoimmune disorder with numerous clinical manifestations, mediated through a thelper 1 response against melanocytes in the eye, inner ear, central nervous system, hair and skin. Some ethnic groups have a higher probability of developing the disease, including asians, indians and latin americans and females are affected more often.
It is clinically diagnosed according to the criteria set by the america uveitis society. Vogtkoyanagiharadalike syndrome definition of vogt. Vogtkoyanagiharada syndrome presenting with encephalopathy. Typically,ocular involvement is a panuveitis associated with choroidal lesions, exudativeretinal detachments, and optic nerve inflammation. Sensorineural hearing loss in vogtkoyanagiharada syndrome. Vogtkoyanagiharada syndrome sharmeen akram and khabir ahmad abstract vogtkoyanagiharada vkh syndrome is a rare multisystem disease of melanocyte containing organs. In 1906, alfred vogt in switzerland first described a patient with premature whitening of eyelashes of.
Two cases of the vogt koyanagi harada syndrome in children, a very rare disorder in this age group, are reported. Vogtkoyanagiharada disease vkh disease is a disease that affects several parts of the body, including the eyes, ears, nervous system, and skin. Vkh may variably also involve the inner ear, with effects on hearing, the skin and the meninges of the central nervous system. The diagnosis is based on clinical and angiographic findings, but some of the characteristic features may be either absent or difficult to visualize in the presence of opaque media. Vkh is a multisystem disorder and includes idiopathic uveitis, dysacousia. Vogtkoyanagiharada vkh syndrome represents a spectrum of bilateral panuveitis with associated central nervous system and dermatologic manifestations. The case of a 62yearold female with diabetes mellitus and a history of primary open angle glaucoma poag in both eyes, operated on the left eye two weeks. The most significant manifestation is bilateral, diffuse uveitis, which affects the eyes. The diagnosis is based on clinical presentation, accounting with a set of standardized diagnostic criteria. Vogt koyanagi harada disease vkh disease is a disease that affects several parts of the body, including the eyes, ears, nervous system, and skin. The disease is characterised by bilateral uveitis associated with a varying constellation of auditory, neurological and cutaneous manifestations. Vogtkoyanagiharada syndrome current perspectives abeir baltmr,1 sue lightman,1,2 oren tomkinsnetzer 1uveitis service, moorfields eye hospital, london, uk.
The vogtkoyanagiharada syndrome vkh is a bilateral, diffuse granulomatous uveitis associated with poliosis, vitiligo, alopecia, and central nervous system and auditory signs. Revised diagnostic criteria for vogtkoyanagiharada disease. Vogtkoyanagiharada syndrome in dogs symptoms, causes. The strong association between vkh disease and certain ethnic groups suggests that genetic factors may be involved. The purpose of this study is to compare the morphological changes of these two diseases with spectral domain optical coherence tomography sdoct.
Vogt koyanagiharada disease vogt koyanagiharada vkh disease,also kwon as uveomeningitic or uveomeningoencephalitic syndrome, is an idiopathic multisystem autoimmune disease. The symptoms of the vogtkoyanagi syndrome overlap those of harada diseasesyndrome, and they may at times be indistinguishable from haradas disease. Vogt koyanagi harada disease vkh is a multisystem disease of presumed autoimmune cause that affects pigmented tissues, which have melanin. Purpose to delineate the historical steps associated with the genesis of the name and the definition of. 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. Fluorescein angiography imaging of a 27yearold male with acute inflammation as part of vogtkoyanagiharada syndrome. The conspicuous feature of the vogtkoyanagi syndrome is the severe, prolonged bilateral uveitis. The most noticeable symptom is a rapid loss of vision. Melanocytes are specialized cells that produce a pigment called melanin. Vkh disease occurs more commonly in patients with a genetic predisposition to the disease, including those from asian, middle eastern.
The term incidence of vogtkoyanagiharada syndrome refers to the annual diagnosis rate, or the number of new cases of vogtkoyanagiharada syndrome diagnosed each year. This condition is common in the third and fourth decade, rarely affecting young children. Once confirmed, although there is no known cure for vogtkoyanagiharada syndrome in dogs, the disease can be kept under control with longterm immunosuppressive medication. The signs and symptoms of vkh disease are caused by chronic inflammation of melanocytes. Vogtkoyanagiharada vkh syndrome is a systemic condition characterized by ocular inflammatory disease as well as skin, ear, and meningeal manifestations. Links to pubmed are also available for selected references. There may also be neurological signs such as severe headache, vertigo, nausea, and drowsiness. The vogtkoyanagiharada syndrome vkh is characterized by bilateral uveitis with serous detachments and signs of systemic disease, including skin disorders alopecia, poliosis, hearing problems loss of hearing, dizziness and signs of meningeal irritation 1,2. Vogtkoyanagiharada syndrome vkh syndrome has neurological, eye, ear, and skin symptoms. Increasing reports have been published to describe the clinical features of vogtkoyanagiharada syndrome in. Vogt koyanagi harada syndrome vkhs is an uncommon systemic syndrome characterized by intraocular inflammation with associated cutaneous, auditory and neurological abnormalitiesl4.
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