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Pathology of Vogt-Koyanagi-Harada Syndrome

Dr Sampurna Roy MD         

 

 

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Syn: Uveomeningoencephalitis Syndrome:   

Vogt-Koyanagi-Harada disease (Syndrome) is a chronic, granulomatous systemic autoimmune disease characterized by ocular inflammatory lesions and extra ocular manifestations in the central nervous, auditory, and integumentary systems. 

The ocular findings of Vogt-Koyanagi-Harada disease may represent a component of a syndrome consisting also of melanoma-associated hypopigmentation. 

Within the framework of current concepts of immunity in patients with cutaneous pigmented malignant melanoma and Vogt-Koyanagi-Harada, the long recurrence-free interval might support the hypothesis of an autoimmune or hypersensitivity process against melanocytes.

It has been suggested by some authors that the use of immunosuppressive therapy in the treatment of VKH and its potential influence on the development of metastatic disease should be carefully reconsidered.

VKH syndrome is characterized by choroiditis, papillitis or neuroretinitis, papilledema and edema of the retina adjacent to the optic nerve in  the posterior uveitis stage, whereas recurrent granulomatous anterior uveitis, "sunset glow" fundus and Dalen-Fuchs nodules  were the common ocular findings in the recurrent anterior uveitis stage.

The common causes of blindness were papillitis, exudative retinal detachment and complicated cataract in the posterior uveitis stage, anterior uveal involvement stage and its recurrent stage.

Typical histopathologic features of Vogt-Koyanagi-Harada disease (VKH) include granulomatous panuveitis with preservation of the choriocapillaris and exudative retinal detachment.

In the chronic stage of the disease, however, histologic changes consist of nongranulomatous uveitis followed, in the chronic recurrent stage, by granulomatous uveitis and involvement of the choriocapillaris.

In chronic VKH the peripheral fundus scars are not Dalén-Fuchs nodules; they are, instead,indicative of focal chorioretinal atrophy with loss of retinal pigment epithelium.

Further reading

Genetic variations of IL17F and IL23A show associations with Behçet's disease and Vogt-Koyanagi-Harada syndrome.

Vogt-Koyanagi-Harada syndrome: A novel case and brief review of focal neurologic presentations.

Fundus abnormalities during the chronic phase of Vogt Koyanagi Harada's disease.

Vogt-Koyanagi-Harada syndrome in children: report of a case and review of the literature.

Vogt-Koyanagi-Harada syndrome in siblings (with a brief review of the literature).

Vogt Koyanagi Harada syndrome. Report of a case with hearing loss, tinnitus, vertigo associated to uveitis.

HLA typing in Vogt-Koyanagi-Harada syndrome in North Indian patients.

Work-up, diagnosis and management of acute Vogt-Koyanagi-Harada disease: a case of acute myopization with granulomatous uveitis.

Vogt-Koyanagi-Harada disease: inquiry into the genesis of a disease name in the historical context of Switzerland and Japan.

Prognostic factors for clinical outcomes in patients with Vogt-Koyanagi-Harada disease treated with high-dose corticosteroids.

 

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Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)


 

 

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