Which of the following causes uveitis with vitiligo and deafness?
ANSWER: (D) Vogt Koyanagi Harada syndromeREF: Kanski 5th Ed Ch 11VOGT KOYANAGI HARADA SYNDROMEVogt-Koyanagi-Harada (VKH) syndrome is an idiopathic multi system autoimmune disease featuring inflammation of melanocyte-containing tissues such as the uvea, ear and meninges.VKH predominantly affects Hispanics, Japanese and pigmented individuals.In different racial groups the disease is associated with HLA-DR1 and HLA-DR4, suggesting a common immunogenic predisposition.In practice, VKH can be subdivided into Vogt Koyanagi disease, characterized mainly by skin changes and anterior uveitis, and Harada disease, in which neurological features and exudative retinal detachments predominate.Possible trigger factors include cutaneous injury or a viral infection which may lead to sensitization of melanocytes.CSF analysis shows pleocytosis with predominant small lymphocytes in about 80% of patients within 1 week and 97% within 3 weeks of disease onset.PHASESProdromal phase lasting a few days is characterized by neurological and auditory manifestations.Meningitis causing headache and neck stiffness.Encephalopathy is less frequent and may manifest with convulsions, paresis and cranial nerve palsies.Auditory features include tinnitus, vertigo and deafness.Acuteuveiticphasefollowssoonthereafterandischaracterizedbybilateralgranulomatous anterior or multifocal posterior uveitis and exudative retinal detachments.Anterior uveitis is usually non-granulomatous during the acute phase but shows granulomatous features during recurrences,which involve only the anterior segment.Posterior uveitis occurs in patients vrith Harada disease and is frequently bilateral. In chronological order the findings are as follows:Diffuse choroidal infiltration and papillitis.Multifocal detachments of the sensory retina and disc oedemaThe chronic phase shows diffuse RPE atrophy (sunset glow fundus) which may be associated with small peripheral atrophic spotsComplications include CNV and subretinal fibrosis.Convalescent phase follows several wreeks later and is characterized by:Localized alopecia, poliosis and vitiligoFocal depigmented fundus lesions (sunset glow fundus) and depigmented limbal lesions (Sugiura sign).Chronic-recurrent phase is characterized by smouldering anterior uveitis with exacerbations.Modified diagnostic criteria for VKH syndrome:1. Absence of a history of penetrating ocular traumaAbsence of other ocular disease entitiesBilateral uveitisNeurological and auditory manifestationsIntegumentary findings, not preceding onset of central nervous system or ocular disease, such as alopecia, poliosis and vitiligoIn complete VKH, criteria 1-5 must be present.In incomplete VKH, criteria 1-3 and either 4 or 5 must be present.In probable VKH (isolated ocular disease), criteria 1-3 must be present.Treatment: involves high-dose oral prednisolone (60-100 mg/day) that maybe augmented with 3-day intravenous pulse therapy with methylprednisolone (500-1000 mg/day). Steroid- resistant patients may require ciclosporin.Prognosis depends on early recognition and aggressive control of the early stages of the disease. Late diagnosis or incorrect initial therapy is more likely to be associated with a guarded prognosis with only 50% of patients having a final visual acuity better than 6/12Option (B): Centurion syndrome is characterized by anterior malposition of the medial part of the lid, with displacement of puncta out of the lacus lacrimalis due to a prominent nasal bridge.Option (C): Alezzandrini syndrome is a very rare disorder characterized by ivhitening of scalp hair, eyebrows, eyelashes & depigmentation of the skin of the forehead, nose, cheek, upper lip, chin all occurring on the same side as unilateral visual changes. There is decrease in visual acuity & an atrophic iris in the affected eye.
Sturge Weber syndrome
Centurion syndrome
Alezzandrini syndrome
Vogt Koyanagi Harada syndrom
Uveal Tract