The diagnosis is based on clinical presentation, accounting with a set of standardized diagnostic criteria. It is important to follow a veterinarians treatment plan. Purpose to delineate the historical steps associated with the genesis of the name and the definition of. The disease is characterised by bilateral uveitis associated with a varying constellation of auditory, neurological and cutaneous manifestations. Two cases of the vogt koyanagi harada syndrome occurring during pregnancy are reported for the first time. 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. Typically,ocular involvement is a panuveitis associated with choroidal lesions, exudativeretinal detachments, and optic nerve inflammation. The classic course of vkh syndrome has three phases. Echographic features of the vogtkoyanagiharada syndrome. Med any combination of signs and symptoms that are indicative of a particular disease or disorder syndrome, mr. Uveomeningoencephalitic syndrome vogtkoyanagiharada. It is an inflammatory condition of autoimmune nature in which cytotoxic t cell target melanocytes in genetically susceptible individuals. Vogtkoyanagiharada syndrome vkh is a systemic inflammatory disorder that predominantly affects individuals with darker skin pigmentation and shows a slight female predilection. Vogtkoyanagiharada syndrome in dogs symptoms, causes.
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. Increasing reports have been published to describe the clinical features of vogtkoyanagiharada syndrome in. The authors used a standard microlymphocyte cytotoxicity assay to type 9 patients with the vogt koyanagi harada syndrome. Links to pubmed are also available for selected references. Vogtkoyanagiharada vkh syndrome represents a spectrum of bilateral panuveitis with associated central nervous system and dermatologic manifestations. Some authors distinguish between the vogtkoyanagi syndrome and the harada disease. Get a printable copy pdf file of the complete article 765k, or click on a page image below to browse page by page. Vogtkoyanagiharada vkh syndrome is a bilateral granulomatous panuveitiswith central nervous system, auditory, and integumentary manifestations. Vogtkoyanagiharada disease genetic and rare diseases. Research suggests it may be due to an autoimmune reaction in which the body attacks its own healthy cells that contain the pigment melanin.
A similar disorder with uveitis and depigmentation of the nose, lips, eyelids, footpads and anus occurs in dogs. 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 vkh syndrome is a rare multisystemic disease of presumed autoimmune etiology, affecting various melanocytecontaining organs. Fluorescein angiography imaging of a 27yearold male with acute inflammation as part of vogtkoyanagiharada syndrome. The signs and symptoms of vkh disease are caused by chronic inflammation of melanocytes. 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 strong association between vkh disease and certain ethnic groups suggests that genetic factors may be involved. Vogtkoyanagiharada syndrome with more emphasis on the. 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. Vogtkoyanagiharadalike syndrome definition of vogt. This casecontrol study develops and evaluates a set of diagnostic criteria for vogtkoyanagiharada disease, an immunemediated disorder. Stephen foster, md introduction vogtkoyanagiharada vkh disease, also known as uveomeningitic syndrome, is an idiopathic multisystem in.
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. The most noticeable symptom is a rapid loss of vision. 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. Vogtkoyanagiharadas syndrome is a rare disease that affects tissues containing melanocytes, such as the eyes, central nervous system, inner ear and skin. 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. Vogt koyanagi harada syndrome vkhs is an uncommon systemic syndrome characterized by intraocular inflammation with associated cutaneous, auditory and neurological abnormalitiesl4. Differential diagnosis of vogtkoyanagiharada syndrome. 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.
Melanin is the substance that gives skin, hair, and eyes their color. Vogtkoyanagiharada vkh syndrome is a systemic condition characterized by ocular inflammatory disease as well as skin, ear, and meningeal manifestations. Development and evaluation of diagnostic criteria for vogt. Vogtkoyanagiharada syndrome sharmeen akram and khabir ahmad abstract vogtkoyanagiharada vkh syndrome is a rare multisystem disease of melanocyte containing organs. Find out information about vogtkoyanagiharada syndrome. Vogtkoyanagiharada syndrome current perspectives abeir baltmr,1 sue lightman,1,2 oren tomkinsnetzer 1uveitis service, moorfields eye hospital, london, uk. Acute vogtkoyanagiharada vkh disease and acute central serous chorioretinopathy cscr are two common disorders with serous retinal detachment caused by dysfunction of choroid. The word symptoms of vogtkoyanagiharada syndrome is the more general meaning.
There may also be neurological signs such as severe headache, vertigo, nausea, and drowsiness. 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. In both, the disease appeared during the second half of pregnancy with complete remission. Recurrent anterior uveitis in patients with vogtkoyanagi. Association between vogtkoyanagiharada syndrome and hladr1 and dr4 in hispanic patients living in southern california. Vogtkoyanagiharada vkh disease is a multisystemic disorder characterized by granulomatous panuveitis with exudative retinal detachments that is often associated with neurologic and cutaneous manifestations. Vkh may variably also involve the inner ear, with effects on hearing, the skin and the meninges of the central nervous system. 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. It is a bilateral granulomatous panuveitis associated with dermatologic and neurologic manifestations, including vitiligo, alopecia, poliosis, and meningeal signs. The aetiology of the disease is still not known although recent findings point to an autoimmune reaction against. We report a case of a 10 year old girl who presented with. The vogtkoyanagiharada syndrome vkh is a bilateral, diffuse granuloma tous uveitis associated with poliosis, vitiligo, alopecia, and central nervous system and auditory signs. The vogtkoyanagiharada vkh syndrome is a rare systemic disorder of uveitis, dysacousia, vitiligo, premature graying of the hair, eyebrows and eyelashes, and meningoencephalitis. 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. Full text full text is available as a scanned copy of the original print version. Vogtkoyanagiharada vkh disease clinical presentation. It is characterized by diffuse granulomatous inflammation involving various organs including eye. Vogtkoyanagiharada syndrome presenting with encephalopathy.
Melanin is the substance that gives skin, hair, and. Vogtkoyanagiharada disease vkh disease is a disease that affects several parts of the body, including the eyes, ears, nervous system, and skin. 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. Vogtkoyanagiharada disease johns hopkins university. Vogtkoyanagiharada disease vkh is a wellestablished multiorgan disorder that affects pigmented structures, such as the eye, inner ear, meninges, and skin 1. 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. 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.
The most significant manifestation is bilateral, diffuse uveitis, which affects the eyes. Neurological involvement typical of vkh syndrome 8 th cranial nerve damage and sterile meningitis. Vogtkoyanagiharada disease nord national organization. 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 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. Alezzandrini syndrome has been closely associated with vogtkoyanagiharada vkh syndrome, as they share similar cutaneous and ocular signs. Two cases of the vogt koyanagi harada syndrome in children, a very rare disorder in this age group, are reported. The conspicuous feature of the vogtkoyanagi syndrome is the severe, prolonged bilateral uveitis. These manifestations are variable and race dependent. Vogtkoyanagiharada syndrome current perspectives opth. The purpose of this study is to compare the morphological changes of these two diseases with spectral domain optical coherence tomography sdoct. Vogtkoyanagiharada disease vkh is a multisystem disease of presumed autoimmune cause that affects pigmented tissues, which have melanin.
Revised diagnostic criteria for vogtkoyanagiharada disease. 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. Comparison of the optical coherence tomographic characters. A case of probable vogtkoyanagiharada disease in a 3yearold. Some ethnic groups have a higher probability of developing the disease, including asians, indians and latin americans and females are affected more often.
We describe a 20yearold britishhonduran man with recent worsening headache and photophobia, vomiting and visual blurring. Although frequently unrecognised,vkh may affect children. In 1906, alfred vogt in switzerland first described a patient with premature whitening of eyelashes of. It is clinically diagnosed according to the criteria set by the america uveitis society. 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 disease vkh is a multisystem disease of presumed autoimmune cause that affects pigmented tissues, which have melanin. The term incidence of vogtkoyanagiharada syndrome refers to the annual diagnosis rate, or the number of new cases of vogtkoyanagiharada syndrome diagnosed each year.
Pdf vogtkoyanagiharada syndrome is a bilateral, chronic, diffuse granulomatous. Vogtkoyanagiharada syndrome vkhs is a rare multisystemic autoimmune disease that affects tissues containing melanin, including the eye, inner ear, meninges, and skin. Vogtkoyanagiharada syndrome vkh syndrome has neurological, eye, ear, and skin symptoms. Vogt koyanagiharada disease vogt koyanagiharada vkh disease,also kwon as uveomeningitic or uveomeningoencephalitic syndrome, is an idiopathic multisystem autoimmune disease.
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. Vogtkoyanagiharada vkh disease is a bilateral granulomatous choroiditis that invariably starts in the choroidal stroma and is therefore called a primary stromal. Vogt koyanagi harada disease vkh disease is a disease that affects several parts of the body, including the eyes, ears, nervous system, and skin. This condition is common in the third and fourth decade, rarely affecting young children. The symptoms of the vogtkoyanagi syndrome overlap those of harada diseasesyndrome, and they may at times be indistinguishable from haradas disease. Indocyanine green angiography guided management of vogt. Sensorineural hearing loss in vogtkoyanagiharada syndrome. Vogtkoyanagiharada syndrome is a granulomatous autoimmune disease involving central nervous system, eyes, skin, and ears.
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