A 31-year-old lady came for routine refractive check-up. Her best corrected visual acuity was 6/6 in both eyes (OU). Intraocular pressure and anterior segment examination were normal in OU. Fundus examination showed clear vitreous, normal disk, retinal vasculature, and fovea. Large ill-defined areas of orange-colored choroidal lesions were noted over superonasal, nasal, and inferonasal quadrants in Right eye (OD) [Fig. 1a]. The rest of the choroidal background appeared normal. Left eye had similar areas of ill-defined orangish choroidal lesions in nasal and inferotemporal periphery [Fig. 1b].
What is Your Next Step?
- Look for similar pigmentary lesions over ocular adnexa or rest of the body
- B scan ultrasonography
- Fundus autofluorescence
- All of the above.
There was no significant previous systemic or ocular history. There was also no history of any topical or systemic medication use in this patient. Thorough examination ruled out cutaneous hypopigmented lesions, poliosis, or white forelock. Ocular examination revealed no evidence of past or present intraocular inflammation. Optical coherence tomography scan through the lesions was normal with no evidence of overlying retinal pigment epithelium (RPE) abnormalities. B scan ultrasonography ruled out mass lesions and revealed normal choroidal thickness throughout. The lesions remained stable in follow-up over a period of 2 years.
Primary choroidal vitiligo.
D. All of the above.
Primary choroidal vitiligo is an uncommon benign asymptomatic lesion that presents as flat depigmentation of the choroid. It is usually associated with cutaneous vitiligo, often of the lids and periorbital region. Secondary choroidal vitiligo occurs following intraocular inflammation, particularly Vogt–Koyanagi–Harada (VKH) syndrome. Choroidal hypopigmentation with uveitis, poliosis, and hyperacusis also constitutes a rare syndrome called Alezzandrini syndrome. Presence of white forelock and iris hypopigmentation with typical facial features points toward Waardenburg syndrome. Investigations including angiography, autofluorescence, and ultrasonography helps us rule out conditions mimicking choroidal vitiligo, including amelanotic choroidal nevus, choroidal osteoma, diffuse choroidal hemangioma, amelanotic melanomas, and chorioretinal atrophy. Usually, primary choroidal vitiligo is a nonprogressive condition and no treatment is required.
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The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
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1. Shields CL, Ramasubramanian A, Kunz WB, Aggarwal E, Shields JA Choroidal vitiligo masquerading as large choroidal nevus:A report of four cases Ophthalmology 2010 117 e3
2. Shields CL, Nickerson SJ, Al-Dahmash S, Shields JA Waardenburg syndrome:Iris and choroidal hypopigmentation:Findings on anterior and posterior segment imaging JAMA Ophthalmol 2013 131 1167 73
3. Alezzandrini AA [Unilateral manifestations of tapeto-retinal degeneration, vitiligo, poliosis, grey hair and hypoacousia] Ophthalmologica 1964 147 409 19