Cas rare de lésions cicatricielles oculaires au cours du Vogt-Koyanagi-Harada

We here report the case of a 27-year old patient, followed-up in our Department for treatment of chronic Vogt-Koyanagi-Harada disease ( VKH disease). Fundus examination showed depigmentation of the retinal pigment epithelium and of the choroid, appearing as a pseudotumoral peripapillary lesion. Vogt-Koyanagi-Harada disease is a multisystem disorder, characterized by bilateral granulomatous panuveitis with serous exudative multifocal retinal detachment. Pathophysiology of this disease is unknown, but an immunological cellular reaction against melanocytes of the skin, the meninges, the retina, the uvea, the cochlea and the labyrinth is suspected. This disease mainly occurs in young subjects from the Far East as well as in pigmented subjects. Ocular involvement is often associated with neurological (meningeal stiffness, headache, sometimes associated with focal deficit and erebrospinal fluid (CSF) pleocytosis), auditory ( perceptive deafness) and cutaneous (vitiligo, poliosis, alopecia and canities) manifestations. It usually evolves in three phases: a prodromal phase mainly characterized by neurological signs, an acute uveitic phase, a chronic phase of convalescence characterized by choroidal and tegument depigmentation or a phase of recurrence during which subretinal neovessels and subretinal fibrosis may appear. Scarrings manifest during the chronic phase of VKH disease, which is dominated by diffuse depigmentation of the fundus of the eye, scars due to nummular chorioretinal atrophy, wheals due to diffuse depigmentation, macular scar remodeling. Pseudotumoral appearance is rare and atypical during the chronic phase of VKH disease. Treatment is based on intravenous corticosteroids followed by a cycle of oral therapy. Patient should be early treated with massive and prolonged therapy to improve prognosis.