Iris Manifestations in Inadequately Treated Chronic Recurrent Vogt-Koyanagi-Harada Disease

Ocul Immunol Inflamm. 2022 Jul;30(5):1116-1122. doi: 10.1080/09273948.2020.1870701. Epub 2021 Feb 9.

Abstract

Purpose: To describe the iris changes in chronic recurrent Vogt-Koyanagi-Harada Disease (VKH).

Methods: Retrospective case series. Clinical features of 3 VKH patients who developed bilateral depigmentation of the iris are described.

Results: Patients had delayed diagnosis and inadequately treated chronic recurrent VKH. Patient 1 presented with bilateral multiple contiguous small granulomatous iris stromal lesions, severe diffuse iris swelling more severe near the iris root, producing peripheral iris undulations. Diffuse iris atrophy and peripheral depigmentation subsequently developed. Patient 2 presented with peripheral iris depigmentation, atrophy of the ciliary zone iris stroma and sparing of the sphincter pupillae, giving a 'sunflower appearance.' Patient 3 had extensive iris depigmentation, atrophy, and transillumination defects involving the entire iris.

Conclusion: Uncontrolled bilateral diffuse granulomatous stromal iris inflammation leads to subsequent depigmentation, transillumination defects and atrophy, beginning in the peripheral iris. Sphincter pupillae is initially spared but complete iris atrophy may develop.

Keywords: Vogt-Koyanagi-Harada disease; iris atrophy; iris depigmentation; iris transillumination defect.

MeSH terms

  • Atrophy / pathology
  • Humans
  • Iris / pathology
  • Iris Diseases* / diagnosis
  • Iris Diseases* / etiology
  • Iris Diseases* / pathology
  • Retrospective Studies
  • Uveomeningoencephalitic Syndrome* / diagnosis
  • Uveomeningoencephalitic Syndrome* / drug therapy
  • Uveomeningoencephalitic Syndrome* / pathology