Síndrome de Vogt-Koyanagi-Harada incompleta associada a HLA DRB1*01 em criança de quatro anos de idade: relato de caso

Abstract

Vogt-Koyanagi-Harada syndrome is chronic systemic autoimmune disease with ocular, nervous, auditory and tegumental manifestations. We report here the case of a child with onset of symptoms at four years and two months of age, with positive HLA DRB1*01.

Síndrome de Vogt-Koyanagi-Harada e o seu acometimento multissistêmico

“…In 2001, the results of this meeting were published by the international committee on nomenclature in the form of revised diagnostic criteria that included: 1-absence of previous history of ocular trauma or surgery; 2-no evidence of other ocular diseases; 3-early bilateral ocular involvement (with focal areas of subretinal fluid or serous retinal detachment) or late bilateral ocular involvement (depigmentation, sunset glow fundus, Dalen-Fuchs nodules and migration or accumulation of the pigmented epithelium of the retina); 4-history or presentation of auditory and/or neurological symptoms; 5-cutaneous symptoms that appear during or after the neurological and ocular manifestations. On the basis of these criteria, VKHS may be classified as complete, when all criteria are met; as incomplete, when criteria 1, 2 and 3 plus 4 or 5 are met, or probable, when only criteria 1, 2 and 3 are met 1,38,40 . Although the criteria for VKHS are well-established, care should be taken when patients are classified, because several factors can affect this classification.…”

“…Also known as uveomeningoencephalitic syndrome, the disease is characterized by chronic bilateral panuveitis associated with a varying constellation of neurological, auditory and cutaneous manifestations 1 . The first descriptions of this disease were written in the 12th century by an Arab physician, Ali IBN ISA, and in the 19th century Jacobi, Nettelship and Tay also described it.…”

“…Children are rarely affected, but when they are the clinical course is more aggressive that in adult cases 6 . The youngest VKHS patient described was a 4-year-old child 1 . The objective of this article is to emphasize the importance of recognizing this syndrome in general clinical practice and in the specialties, with a focus on its etiology and pathogenesis, clinical manifestations and the many different forms of treatment that are currently proposed.…”

Síndrome de Vogt Koyanagi Harada com manifestação cutâneo vulvar: relato de caso

“…Vale lembrar de suas múltiplas facetas e apresentações, sendo de suma importância frisar, neste caso, a fase crônica da doença, que é marcada pelo processo de despigmentação de vários tecidos que contém melanócitos, como olhos e pele. Dois a três meses após o início dos sintomas é possível evidenciar as alterações cutâneas como a poliose, vitiligo e alopecia, e que podem permanecer por meses a anos (LUCENA DR, et al, 2007).…”

“…A Síndrome de Vogt-Koyanagi-Harada (SVKH) é uma patologia pouco frequente, multissistêmica e que envolve tecidos com melanina. Ela também é conhecida como síndrome uveomeningoencefálica, caracterizada por uma panuveíte bilateral crônica, em conjunto com alguma manifestação sistêmica variável de comprometimento neurológico, auditivo e cutâneo (LUCENA DR, et al, 2007). A doença foi descrita a primeira vez por um médico árabe, Ali IBN ISA, no século XII e, mais tarde, no século XIX por Jacob, Nettelship e Tay.…”