Neurosarcoidosis Occurring 6 Years After Onset Of Vogt-Koyanagi-Harada Disease

A 43-year-old Japanese woman visited a clinic with a 2-week history of visual loss, hyperemia, and bilateral eye pain. Dilated fundus and optical coherence tomography showed hyperemic optic disc and multifocal serous retinal detachments. Fundus fluorescein angiography revealed optic disc leakage and numerous hyperfluorescent pinpoints of leakage, which are typical of Vogt-Koyanagi-Harada disease (VKHD). She also showed tinnitus, poliosis, and alopecia. With a diagnosis of VKHD, steroid therapy was started. At the age of 48, oral prednisolone was tapered off, and only corticosteroid eye drops were continued. Eighteen months later, dysesthesia appeared in the left side of her face and in her left ring and little fingers, which spread to the ulnar side of her forearm. Blood tests indicated elevated serum angiotensin-converting enzyme (ACE) levels. Chest CT showed bilateral lymphadenopathy, and histological analysis of the subcutaneous mass in her right arm showed multinucleated giant cells. Steroid therapy was started under a diagnosis of sarcoidosis. Serum ACE and lysozyme levels decreased in response to steroid therapy. We report a case of the concurrence of VKHD and sarcoidosis, which may share a common pathophysiology. Accumulation of further similar cases is necessary to elucidate the precise mechanism underlying the concurrence of these two diseases. (C) 2019 The Author(s) Published by S. Karger AG, Basel