Author Affiliations: The Eye Center, The Eye Foundation for Research in Ophthalmology, and Department of Ophthalmology, College of Medicine, King Saud University, Riyadh, Saudi Arabia; and Wilmer Eye Institute, Johns Hopkins University, School of Medicine, Baltimore, Maryland.
Vogt-Koyanagi-Harada (VKH) disease is a chronic multisystem disorder characterized by an acute onset.1- 4 The disease may be associated with signs of meningeal irritation and may later develop integumentary signs of poliosis and vitiligo that are valuable in the diagnosis of the disease. Poliosis and vitiligo occur as late clinical manifestations of VKH disease and help in making the diagnosis of complete VKH disease.2 Reversal of the poliosis and vitiligo has not been previously emphasized. We report reversal of poliosis and vitiligo among patients with VKH disease.
The study was approved by the institutional review board at The Eye Center, Riyadh, Saudi Arabia. A total of 22 patients with VKH disease were included. Patients had complete VKH disease as defined by the International Committee on Nomenclature.2 There was no history of penetrating trauma or ocular surgery preceding the onset of the disease. Patients were followed up for a mean of 8 years (range, 4-18 years). The presence of poliosis and vitiligo was assessed, and all patients were evaluated for activity of the disease.3
All patients were treated with prednisone, 1 mg/kg/d, for 3 months. Patients were also treated with one of the following immunosuppressive agents for a minimum of 6 months: azathioprine sodium, methotrexate sodium with folic acid, or mycophenolate mofetil. Reversal of poliosis and vitiligo and relapse of active intraocular inflammation were the outcome measures. A relapse was defined as a flare-up of disease activity 3 or 4 months after a remission.5,6
For overall analysis, SAS version 9.2 statistical software (SAS Institute, Inc) was used. Patient characteristics were compared using the Wilcoxon rank sum test or Fisher exact test. Differences in medians were tested with the nonparametric Wilcoxon rank sum test.
There were 22 patients (16 females and 6 males). The mean age was 28 years, and the age range was 5 to 58 years. All patients had vitiligo and poliosis. Reversal of vitiligo and poliosis was complete and was noted in 6 patients at a mean follow-up of 19.6 months (range, 18-30 months) following onset of the disease (Figure 1 and Figure 2). The mean (SD) age was 15.3 (7.9) years for patients with reversal of vitiligo and poliosis compared with 34.0 (8.2) years for patients who had no reversal (P = .004) (Table). The median age was 14.5 years for those with reversal and 32.5 years for patients without reversal. None of the 6 patients who had reversal of their poliosis and vitiligo had relapse of uveitis at 30 months compared with 7 of the 16 patients who had no reversal of poliosis or vitiligo (P = .04). Systemic steroids were given for longer periods in patients without reversal compared with patients with reversal of vitiligo and poliosis (P = .007). Nominal P values are provided for all comparisons in the Table.
Figure 1. The left upper eyelid with poliosis of the eyelashes 6 weeks after the acute onset of complete Vogt-Koyanagi-Harada disease in a 14-year-old patient.
Figure 2. Total reversal of poliosis of the left upper eyelid eyelashes in the same patient shown in Figure 1.
The skin changes are important diagnostic features of VKH. In this series of patients, however, there was reversal of vitiligo and poliosis in patients with complete VKH disease.
The reversal of poliosis, vitiligo, and alopecia in patients with VKH has not been previously emphasized. Reversal of poliosis and vitiligo should be taken into consideration in the diagnosis of VKH disease. Following a period of immunologic dysregulation in VKH disease, reversal of poliosis and vitiligo may suggest restoration of the normal immune homeostasis.
All patients with reversal of the poliosis and vitiligo had no intraocular inflammation. The reversal of poliosis and vitiligo may indicate a good prognostic sign and may indicate remission of the disease.
Correspondence: Dr Tabbara, The Eye Center, 241 Makkah Rd, PO Box 55307, Riyadh 11534, Saudi Arabia (firstname.lastname@example.org).
Financial Disclosure: None reported.
Funding/Support: This work was supported in part by a grant from The Eye Foundation for Research in Ophthalmology and The Eye Center, Riyadh, Saudi Arabia.
Additional Contributions: Naser Elkum, PhD, Department of Biostatistics and Epidemiology, Dasman Diabetes Institute, Dasman, Kuwait, assisted in the statistical analysis.
Thank you for submitting a comment on this article. It will be reviewed by JAMA Ophthalmology editors. You will be notified when your comment has been published. Comments should not exceed 500 words of text and 10 references.
Do not submit personal medical questions or information that could identify a specific patient, questions about a particular case, or general inquiries to an author. Only content that has not been published, posted, or submitted elsewhere should be submitted. By submitting this Comment, you and any coauthors transfer copyright to the journal if your Comment is posted.
* = Required Field
Disclosure of Any Conflicts of Interest*
Indicate all relevant conflicts of interest of each author below, including all relevant financial interests, activities, and relationships within the past 3 years including, but not limited to, employment, affiliation, grants or funding, consultancies, honoraria or payment, speakers’ bureaus, stock ownership or options, expert testimony, royalties, donation of medical equipment, or patents planned, pending, or issued. If all authors have none, check "No potential conflicts or relevant financial interests" in the box below. Please also indicate any funding received in support of this work. The information will be posted with your response.
Some tools below are only available to our subscribers or users with an online account.
Download citation file:
Web of Science® Times Cited: 1
Customize your page view by dragging & repositioning the boxes below.
Enter your username and email address. We'll send you a link to reset your password.
Enter your username and email address. We'll send instructions on how to reset your password to the email address we have on record.
Athens and Shibboleth are access management services that provide single sign-on to protected resources. They replace the multiple user names and passwords necessary to access subscription-based content with a single user name and password that can be entered once per session. It operates independently of a user's location or IP address. If your institution uses Athens or Shibboleth authentication, please contact your site administrator to receive your user name and password.