F16 VOGT-KOYANAGI-HARADA-SYNDROME
Barraud-Klenovsek M, Senti G, Trüeb RM
Department of Dermatology, University Hospital of Zurich, Switzerland.

A 46-year-old female patient was referred from the Department of Ophthalmology. Her symptoms had begun 1½ years previously, when she developed severe headaches shortly after having suffered a common cold. This was followed by persistent vertigo, and visual impairment, such as diplopia, progressive loss of visual acuity, and painful eyes. Later she experienced progressive bilateral hearing loss associated with tinnitus. During this period, she lost her left upper eyelashes, followed by regrowth of white lashes. Skin examination revealed poliosis of the left upper eyelashes, but no other pigmentary abnormalities. She was fitted with bilateral hearing aids. She was also investigated by neurologists who confirmed her initial symptoms to be compatible with meningitis, and excluded an infectious cause. An MRI of the skull at onset of symptoms was normal. The differential diagnosis of poliosis (especially of the ocular region) associated with ocular and/ or auditory symptoms includes albinism, piebaldism, Waardenburg syndrome, Woolf syndrome, Vogt-Koyanagi-Harada (VKH) syndrome, and Alezzandrini syndrome. The adult onset as well as the characteristic progression of symptoms following a mild viral disease are in favour of the VKH syndrome, and practically exclude the other diagnoses mentioned. VKH syndrome is rare, affects women more frequently than men, as well as darker pigmented individuals. The typical course consists of 3 phases (meningoencephalitic, ophthalmic-auditory, and a convalescent phase). The cutaneous manifestations develop during the last phase, and include poliosis in 90% of patients, alopecia areata, vitiligo, and halo naevi. The etiology remains unclear, but data support an autoimmune reaction directed against uveal, dermal and meningeal melanocytes. The American Uveitis Society has recommended diagnostic criteria. Treatment is determined by the severity of the ocular changes, and consists of systemic corticosteroids and other immunosuppressive agents.