The patient had recurrent histories of remission and relapse with encephalomyelitis, there were periventricular and pontine hyperintense areas in t. However, combined steroid and azathioprine was the most commonly used regimen in the present report 1419 cases. The following criteria are required to make a diagnosis of vkh syndrome. 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. Concurrent presence of retinal hemorrhages in the setting. Editor,vogtkoyanagiharada vkh syndrome is a bilateral, diffuse, granulomatous uveitis associated with exudative retinal detachment and central nervous system, auditory, and dermatological involvement. Uveodermatological syndrome 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 syndrome vkh is a disease distinguished by a triad of uveitis, dermatologic findings, and cns involvement. Arthritis and inflammatory eye disease rheumatology. Dear editor, vogtkoyanagiharada disease vkh is an autoimmune disorder affecting melanocytes.
Vogtkoyanagiharada vkh disease is defined as a bilateral granulomatous panuveitis with or without extraocular. The inflammation lasts several weeks, and early treatment may prevent the later. Vogtkoyanagi harada disease is a multisystem disease of presumed autoimmune cause that affects pigmented tissues, which have melanin. Day syndrome, diabetes, amyloidosis syndrome, or paraneoplastic syndrome, is suggested. Publishing results from original hypothesisbased clinical and laboratory ophthalmic and vision research studies. Vkh syndrome almost always affects more heavily pigmented individuals, and is seen in women more than twice as often as men, typically in the third and fourth decades of life. During her 24month followup, the patient did not present any recurrence. Vogtkoyanagiharada syndrome nattaporn tesavibul, m. Vogtkoyanagiharada disease vkh is a multisystem disease of presumed autoimmune. Leading clinical and laboratory ophthalmic and vision research. Focal neurologic signs such as cranial nerve palsies, hemiparesis, and optic neuritis are relatively uncommon. Managing macular hole associated with acute inflammatory vogt koyanagi harada syndrome rodrigo m. Bilateral iritis, vitritis, and choroidal thickening were evident. Neurologic manifestations of vkh typically include aseptic meningitis and headache.
Angle closure glaucoma as a presenting sign of vogt. 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. Gender differences in vogtkoyanagiharada disease and. Vogtkoyanagiharada syndrome vkh is a rare inflammatory disorder diagnosed clinically that presents as panuveitis with serous retinal detachments among other systemic symptoms. Recurrent unilateral vogtkoyanagiharada disease with. Later similar cases were reported by koyanagi in 1929,3 after which the entity was identified as vogtkoyanagi syndrome. Initiation of prompt and appropriate treatment prevents blindness and other complications. It is also often associated with skin, auditory and neurologic manifestations. The criteria of the international study group for bd and first international workshop for vkh were set as the diagnostic criteria for the diagnosis of bd and vkh syndrome respectively 52,53. Infectious aetiology has been proposed which is suggested to lead to the loss of melanocytes in the skin, inner ear, meninges, and uvea in those who are genetically predisposed. This disease is mainly a th1 lymphocyte mediated aggression to melanocytes after a viral trigger in the presence of hladrb10405 allele. Vogtkoyanagiharada syndrome associated with psoriasis.
She was diagnosed with vogtkoyanagiharada disease based on her clinical. The vogtkoyanagiharada vkh syndrome is a multisystem disease characterized by acute bilateral panuveitis. 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 vogtkoyanagiharada vkh syndrome is a multisystemic immune disorder. Vogtkoyanagiharada syndrome current perspectives abeir baltmr,1 sue lightman,1,2 oren tomkinsnetzer 1uveitis service, moorfields eye hospital, london, uk.
It predominantly affects asian people, but also people with darker skin pigmentation such as. Vkh classically has been reported as more prevalent in females than males, yet some studies in japan and china have not found differences in gender. Named after the three scientists who detected this disease in humans. The academy uses cookies to analyze performance and provide relevant personalized content to users of our website. Vogtkoyanagiharada disease, female, posterior uveitis, retinal detachment. Vogtkoyanagiharada disease vkh disease is a disease that affects several parts of the body, including the eyes, ears, nervous system, and skin. Labelfree proteomics reveals decreased expression of cd18. Melanocytes are specialized cells that produce a pigment called melanin. The clinical presentation and diagnosis of vogtkoyanagi. Vogtkoyanagiharada syndrome vkh is a bilateral granulomatous panuveitis associated with cutaneous poliosis, alopecia, and vitiligo, neurological aseptic meningitis and auditory dysacusis, tinnitus, vertigo manifestations related to a cellmediated autoimmune process against melanocytes 1. Note the smoothing out of the choroidal undulations with treatment. Managing macular hole associated with acute inflammatory. Editor,vogtkoyanagiharada vkh syndrome is an uncommon disorder characterised by panuveitis in association with neurological and cutaneous abnormalities including headache, tinnitus, vitiligo, poliosis, and alopecia. Vkh background vkh syndrome or uveomeningitic syndrome is a systemic disorder involving many organ systems, including the eye, ear, integumentary and nervous system.
Machado2 and mauricio maia1,2 abstract we report a 24yearold man with vogtkoyanagiharada vkh syndrome who developed a macular hole mh during the acute inflammatory stage. It predominantly affects hispanic, japanese and pigmented individuals. The most significant manifestation is bilateral, diffuse uveitis, which affects the eyes. Early diagnosis of acute, purely ocular vkh disease is crucial for prompt initiation of corticosteroid treatment to ensure a good visual outcome and to prevent chronicrecurrent inflammation and. 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. We report a case of vkh syndrome with an unusual presentation of. Information regarding vkh syndrome is scanty among the african population. The exact cause of vogtkoyanagiharada 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 vkh disease is defined as a bilateral granulomatous panuveitis with or without extraocular manifestations affecting young adults. Dear editor, vogtkoyanagiharada vkh disease usually presents as bilateral panuveitis associated with poliosis, vitiligo, alopecia, and central nervous system and auditory signs. Schachat ap ss, hinton dr, wilkinson cp, wiedemann p, editor. Optical coherence tomography angiography in incomplete acute vogtkoyanagiharada disease. The absence of ocular trauma or previous intraocular surgery sets vkhd appart. Dr olivier calvetti dr caroline laurentcoriat pr michel. Patients are normally pigmented individuals of both sexes. Vogtkoyanagiharada syndrome vkh is a bilateral granulomatous panuveitis associated with cutaneous, neurological and auditory manifestations 1. Vogtkoyanagiharada syndrome vkh is a very uncommon hypomelanotic disorder affecting melanocytes of the skin, ocular, auditory and central nervous system cns. Full text vogtkoyanagiharada syndrome current perspectives. Evolving understanding and treatment of vogtkoyanagiharada. A 50yearold woman presented with conjunctival injection and bilateral eye pain. It is one of the most common uveitis entities in chinese as well as in individuals of americanindian descent.
Vogtkoyanagiharada vkh syndrome is a multisystemic autoimmune disease of uncertain pathogenesis. The immune response in vkh syndrome most likely is directed against antigens associated with melanocytes present in all organ systems, including the choroid. Vkh may variably also involve the inner ear, with effects on hearing, the skin and the meninges of the central nervous system. Traf5 and traf3ip2 gene polymorphisms are associated. Vkh syndrome is a multisystem autoimmune disorder directed against melanocytes and is characterized by bilateral granulomatous uveitis with meningeal irritation sign mis, dysacusis, vitiligo, poliosis and alopecia 10. A suggested practical diagnostic approach to vkh disease, taking into account the chronological phase of the disease may be helpful box 2. Treatment and visual prognosis mumtaz alam, mustafa iqbal, bakht samar khan and ibrar hussain abstract vogt koyanagi harada vkh disease is a chronic, bilateral, granulomatous panuveitis associated with central nervous system, auditory and. Methylprednisolone iv 40 mgday for 3 days followed by prednisone p. It was first described by an persian physician aliibnisa 9401010a. Vkh disease occurs more commonly in patients with a genetic predisposition to the disease, including those from asian, middle eastern.
In 1906, alfred vogt in switzerland first described a patient with premature whitening of eyelashes of sudden onset and bilateral subacute. Vogtkoyanagiharada disease vkh and sympathetic ophthalmia so are types of tcell mediated autoimmune granulomatous uveitis. In unilateral cases, the second eye is typically affected within 2 weeks of the first eye. Bhupinder johala, herman johala, andrew lukaris full text pdf, 182kb abstract. Complete vogtkoyanagiharada disease and holmes adie.
Vogtkoyanagiharada disease vkhd is a rare granulomatous inflammatory disease that affects pigmented structures, such as eye, inner ear, meninges, skin and hair. The use of corticosteroids and immunosuppressive agents has greatly improved the visual outcome in vkh patients. Vogtkoyanagiharada vkh syndrome is a systemic autoimmune disease. Vision was 20400 and 2080 in the right and left eyes, respectively. Clinical spectrum and management options in vogtkoyanagiharada disease sikander ak lodhi, jm lokabhi reddy, venkataratnam perum department of ophthalmology, osmania medical collegesarojini devi eye hospital, hyderabad, telangana, india purpose. Information about vogtkoyanagiharada syndrome vkh syndrome vogt koyanagi harada syndrome vkh syndrome is a condition seen in humans and dogs involving various melanocytecontaining organs, characterized by uveitis inflammation of the inside of the eye, poliosis whitening of hair, vitiligo loss of pigment in the skin, and. Full text clinical spectrum and management options in. First recognized at the beginning of twentieth century and named after three authors who independently described some affected patients, vogtkoyanagiharada syndrome is a rare multisystemic autoimmune disease targeting melanincontaining tissues of the eye, meninges, inner ear and skin. Initial aggressive treatment with ivmp, peribulbar longacting corticosteroids, and. Vogtkoyanagiharada disease, diabetes mellitus, and. Vogtkoyanagi harada syndrome is a cause of noninfectious panuveitis. Vkh syndrome is an idiopathic multisystem autoimmune disease featuring inflammation of melanocyte containing tissues such as the uvea, skin, ear, and meninges.
Vogtkoyanagiharada vkh disease is characterized as a bilateral. Although the two diseases share common clinical features, they have certain differences in gender predilections. This article is featured in a journal club episode of straight. Increased peripheral blood cd4plus cd8plus tcell ratios have been seen in vkh, suggesting an. Vogtkoyanagiharada vkh disease is a multisystem autoimmune inflammatory disorder with ocular, auditory, skin and neurologic involvement. Vogtkoyanagiharada disease zielinski family home page. Topographic distribution of contractile protein in the human. Patients may present with a polyarthritis but the organs that contain melanocytes are targets of inflammation, resulting in areas. F q 2 h ou cyclo 1% tid ou ranitidine 150 mg referred to rheumatology. Melanin is the substance that gives skin, hair, and eyes their color. The signs and symptoms of vkh disease are caused by chronic inflammation of melanocytes. 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 vogt koyanagiharada vkh syndrome is a multisystemic immune disorder. Melanin is what gives color, or pigment, to our hair, skin and parts. Vogtkoyanagiharada syndrome vkh, an autoimmune disorder, is an oculocutaneous meningeal syndrome commonly seen in pigmented race population characterized usually by bilateral granulomatous panuveitis, often associated with neurological, auditory, and cutaneous manifestations.
Vogtkoyanagiharada vkh disease department of ophthalmology. With the criteria met,1 the diagnosis of complete vogtkoyanagiharada syndrome was also supported. Vogtkoyanagiharada vkh syndrome or uveoencephalitis is a rare systemic disease of melanocytecontaining organs. Vogtkoyanagiharada syndrome vkh is a bilateral granulomatous panuveitis associated with serous retinal detachments and vitritis, and can be associated with extraocular manifestations of meningismus, poliosis, vitiligo, hearing loss, and headaches. Vogtkoyanagiharada disease genetic and rare diseases. In 1906, alfred vogt in switzerland first described a patient with premature whitening of eyelashes of sudden onset and. Vogtkoyanagiharadalike syndrome is an immune mediated disease with severe autoaggression against melanocytes that are the pigment producing cells of the skin and eyes iris. In 19 casecontrol studies investigating the association of hladr4hladrb104 with vkh, the ors of individual studies ranged from 1. We read with a special interest the article by helvestone and gilmore 1 that described a case with uveitis, poliosis, and recurrent encephalomyelitis without meningitis diagnosed as a vogtkoyanagiharada syndrome vkh.
The incidence of vkh among singaporean population in referral care centre was found to be 3%. Vogtkoyanagiharada syndrome vkh is a multisystemic granulomatous autoimmune disease affecting organs with high melanocyte concentrations including the eye, cns, inner ear, and skin. The aim of this study was to describe the clinical features, treatment options, and visual outcome of vogtkoyanagiharada vkh disease. Case report a 52yearold female presented with complaints of headache, decreased vision, and redness in both eyes for 1 week.
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