Tonic Pupils in a Young Female with Vogt Koyanagi Harada Disease

Introduction

Vogt-Koyanagi-Harada (VKH) disease is an inflammatory disorder attributed to an autoimmune reaction against tyrosinase-like proteins in melanocytes in the uvea, the inner ear, the meninges, and the integumentary system [1]. VKH mainly manifests in people with higher melanin levels (e.g., Asians, Asian Indians, Hispanics, and American Indians) [2], [3], and less often in Caucasians [4]. At the onset of the disease, the first anatomical structure to be affected is the melanocyte islets in the choroidal stroma [5]. The inflammatory process starts as choroiditis [6], which evolves to granulomatous panuveitis with exudative retinal detachments. Sometimes, auditory and meningeal symptoms may also develop [2], with mononuclear pleocytosis in the cerebrospinal fluid [7], [8]. Commencing patients on prompt and vigorous immunosuppressive treatment is crucial to prevent uveitis from becoming chronic and protect the integumentary and auditory systems [8]. Interestingly, in some rare cases, the tonic pupil has been described in patients with VKH syndrome [9], [10], [11], [12], [13]. Apart from ocular inflammation, tonic pupil can develop in several conditions including peripheral or autonomic neuropathy. Adieʼs tonic pupil, also known as Adie Syndrome or Holmes-Adie Syndrome, is defined by a poor pupillary light reflex, strong pupillary response to near stimuli, slow redilation of the pupil after constriction to near stimuli, and accommodation paresis. This disorder is caused by the damage postganglionic parasympathetic innervation of the intraocular muscles [14]. Herein, with this report, we present a case of a young female patient who presented with bilateral tonic pupils associated with VKH syndrome.

Publication History

Received: 04 February 2025

Accepted: 23 April 2025

Article published online:
28 May 2025

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