Bilateral asymmetrical choroidal vitiligo

A 50-year-old woman of Indian origin was referred to us to rule out choroidal melanoma in both eyes. She was asymptomatic, and there was no history of intraocular inflammation, trauma, or any systemic illnesses. On examination, her best corrected Snellen's visual acuity was 20/20 in both eyes (BE) with a refractive correction of +0.5D. Anterior segment examination was unremarkable with brown color irides in BE [Figure 1a and b]. Wide-field fundus imaging of the right eye (RE) revealed choroidal hypopigmentation of the nasal half of the fundus with its irregular border passing temporal to the fovea and extending to the inferior and superior periphery measuring 35 mm in diameter. The left eye (LE) fundus revealed choroidal hypopigmentation with ragged irregular margins present only in the superonasal quadrant, with a small linear extension toward the optic disc measuring 7 mm in diameter [Figure 2a and b]. Short-wavelength fundus autofluorescence (FAF) revealed normal foveal hypoautofluorescence in BE [Figure 2c and d]. Spectral-domain optical coherence tomography (SD-OCT) of BE revealed a normal foveal contour with a normal retinal pigment epithelium, and an increased patchy choroidal hypertransmission was seen between the fovea and nasal macula in BE. Additionally, SD-OCT in the RE revealed mild choroidal thinning in the region of depigmentation [Figure 2e and f]. Detailed physical examination did not reveal any cutaneous hypopigmentation or white forelock [Figure 1-e]. Based on history, systemic examination, and multimodal imaging, she was diagnosed with BE asymmetric choroidal vitiligo and was advised observation and close follow-up.

Figure 1.

Anterior segment photograph of the RE and LE, respectively, revealing a normal iris color and pattern (a and b); no cutaneous vitiligo or white forelock seen (c-e)

Figure 2.

Color fundus photographs of the RE and LE, respectively, showing the asymmetric choroidal hypopigmentation seen more extensively in the right eye involving the macula. The LE shows sectoral choroidal hypopigmentation in the superonasal quadrant (a and b); fundus autofluorescence of BE showing normal foveal hypoautofluorescence (c and d); spectral-domain optical coherence tomography of BE showing a normal foveal contour. An increased patchy choroidal hypertransmission was seen between the fovea and nasal macula in BE. Note the choroidal thinning in the area of hypopigmentation in the RE (arrowheads). An increased patchy choroidal hypertransmission was seen between the fovea and nasal macula in BE. (e and f)

Primary acquired choroidal vitiligo manifests as flat hypopigmentation of the normally pigmented choroid and occurs as an idiopathic condition without any preceding inflammation.[1] This condition, although rare, has been described previously in case reports to be associated with cutaneous vitiligo, with the pathology being the destruction of the choroidal and cutaneous melanocytes which are both derived from the neural crest.[1,2,3,4] Previous reports have described the choroidal hypopigmentation to be symmetrical or asymmetrical between the eyes, occurring along a sector of the fundus, presenting as patchy areas of hypopigmentation, and occurring in an X-shaped pattern.[1,2,3,4] Multimodal imaging findings have further revealed a normal retinal architecture on OCT and normal retinal pigment epithelial function on FAF, although scleral hyperautofluorescence was noted in the region of the depigmented choroid and mild choroidal hyperfluorescence in the region of vitiligo on fundus fluorescein angiogram.[1]

Wibbelsman et al.[5] reported symmetrical sectoral choroidal hypopigmentation along with multimodal imaging findings in a Caucasian woman without cutaneous vitiligo or white forelock. Similar to their case, we describe the multimodal imaging findings of an Indian woman with choroidal hypopigmentation without cutaneous vitiligo. She did not have a history of uveitis, tinnitus, headache, meningismus, progressive vitiligo, or rheumatological symptoms. Unlike the case reported by Wibbelsman et al.,[5] a gross asymmetry in the pattern and extent of choroidal hypopigmentation was seen in the present patient with extensive involvement in the RE and only a sectoral involvement in the LE. Visual function was preserved in the present patient with a normal retinal architecture on SD-OCT, although mild choroidal thinning was noted at the region of hypopigmentation. FAF in the present patient did not reveal scleral hyperautofluorescence. Nevertheless, this is the first report of asymmetrical choroidal vitiligo seen in an Indian woman without associated cutaneous vitiligo and this may represent either an early sign or a limited form of vitiligo.

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