Clinical and histopathologic observations in severe Vogt-Koyanagi-Harada syndrome

Vogt-Koyanagi (V-K) syndrome is a severe anterior uveitis associated with alopecia, vitiligo, poliosis, and dysacousia. Harada's disease (HD) is primarily a posterior uveitis accompanied by signs of meningeal irritation and abnormalities of the cerebrospinal fluid. The overlapping of clinical manifestations between the two justified considering them as part of a spectrum of one disease (V-K-H). In a clinicopathologic study of nine cases four patients (three with V-K syndrome and one with V-K-H syndrome) histopathologically displayed a granulomatous uveitis. Five patients (two with V-K syndrome and three with HD) showed a nongranulomatous uveitis. We emphasized this latter finding, since in the past the diagnosis of this syndrome has been discarded both clinically and histopathologically because of the absence of a granulomatous uveitis. We established the clinical and histopathologic differences between V-K-H and sympathetic ophthalmia. The designation "uveomeningoencephalitic syndrome" stresses the key features of V-K-H, namely the uveal involvement and signs of meningeal irritation (that is, headaches, personality changes, and cerebrospinal fluid alterations).