Vogt-Koyanagi-Harada Disease in which Poliosis and Alopecia Occurred after a Long Period of Time.

Abstract

Vogt-Koyanagi-Harada (VKH) disease is a multisystem autoimmune disorder affecting melanocyte-containing tissues such as the eyes, ears, central nervous system, and skin. A 55-year-old Japanese male presented with an 8-week history of increasing diffuse hair loss. A physical examination revealed diffuse alopecia of the scalp. He lost all body hair, including the eyebrows and eyelashes. All the remaining scalp hair was white, but no vitiligo was present. Laboratory examination found nearly normal values and the HLA profile was DR4. The patient was initially diagnosed with alopecia universalis and received 15 intradermal injections of 1 mg of triamcinolone acetonide to the same region. As a result, although no hair growth was observed, the patient's hair turned black in the area of the local injections (Figure 1, a). We therefore reviewed the patient's medical history again and found that he had developed bilateral uveitis at the age of 20 years and received high-dose intravenous corticosteroid therapy. He also consulted an ophthalmologist at the time, but there were no abnormal findings. Two biopsies were performed in both the white and black hair. Histopathologically, no melanin was detected in the white hair (Figure 1, b), whereas the black hair demonstrated melanin granules in the hair matrix (Figure 1, c). Treatment was started with oral prednisolone at a dose of 30 mg per day, with the dose gradually reduced by 5 mg every 2 weeks. When the treatment was discontinued, a considerable part of the hair had turned black (Figure 1, d). VKH disease typically has three phases. During the first phase, patients may experience headache, muscle weakness, and meningism. Recovery is usually complete, and is followed by the second phase days to weeks later, in which patients may develop uveitis, iridocyclitis, choroiditis, and dysacousia. The third phase begins weeks to months later as the uveitis improves. The third phase is the convalescent phase, and is characterized by alopecia, poliosis, and vitiligo; usually occurring in that order. These dermatologic manifestations have been reported in about 50% of patients by two months after disease onset (1). Poliosis is either patchy or diffuse and occurs in up to 90% of the patients (2). In our case, the amount of white hair began to increase 20 years after the onset of uveitis, and all the hair turned white over the course of a few years. Such a late onset of poliosis has not been reported so far, and steroid treatment resulted in repigmentation even approximately 15 years after all the hair turned white. Reversal of poliosis and vitiligo in the VKH setting has been reported in 6 of 22 (27%) patients (3). All patients with reversal of poliosis and vitiligo had no intraocular inflammation. The reversal may represent a good prognostic sign. Our patient also experienced no recurrence of ocular symptoms. Alopecia can be observed in 50%-70% of cases of VKH disease and occurs a few weeks to a few months after onset of ocular or meningeal symptoms (4). It presents as diffuse, although it may be patchy. Alopecia usually heals after a variable period of time with steroid or immunosuppressive therapy. Microscopically, alopecia in VKH disease shows a peribulbar mononuclear infiltrate with increased telogen/catagen/anagen follicles (2). So far, no reports have compared the histopathological findings of white and black hair in the same patients. No inflammatory infiltrate was present in our case. The reason for this may be the chronic phase of the disease. Our patient developed alopecia 35 years after the onset of uveitis, and such a late onset of alopecia as well as poliosis has not been reported so far. Unfortunately, we have observed no growth of scalp hair. The patient continues to attend follow-up, with special attention to the potential onset of vitiligo.