Ocular Abnormalities in Vitiligo Patients: A Cross-Sectional Study.

Sir,

Vitiligo is an acquired discoloration of the skin caused by epidermal melanocyte loss or damage. Various studies have found the association of ocular abnormalities with vitiligo.[1234567] We studied 52 clinically diagnosed vitiligo patients. Pregnant females, patients with history or evidence of ocular trauma, and systemic diseases such as diabetes, hypertension etc. were excluded. A complete ophthalmologic examination was conducted by an ophthalmologist. Detailed ocular examinations included best corrected visual acuity assessment, refraction, external examination with torchlight and slit lamp biomicroscopy, gonioscopy, and dilated fundus examination with 90 D lens. The data was summarized using descriptive statistics, frequencies, and percentage. Statistical differences between categorical variables were assessed using the Chi-square test. A P value <0.05 was considered statistically significant.

There were 28 males and 24 females in the study. The mean age of the patients was 26.7 years ranging from 7 years to 60 years. The mean age of onset of vitiligo was 21.7 years ranging from 4 years to 50 years. Eight patients (15.3%) had a positive family history and included first-degree relatives in 4 (7.6%), second-degree relatives in 3 (5.7%) patients, and both first and second degree relatives in 1 (1.9%) patient. The majority of [51 (96%)] patients had non-segmental vitiligo. Vitiligo vulgaris was the most common (36.5%) clinical type followed by acrofacial (19.2%), facial (17.3%), acral (13.5%), focal (5.8%), vitiligo universalis (3.8%), mucosal (1.9%), and segmental (1.9%). The usual site of onset was head and neck in 22 (42.3%), lower limbs in 12 (23%), upper limbs in 9 (17.3%), trunk in 6 (11.5%), and mucosa in 3 (5.8%) patients. Body surface area involvement was <50% in 50 (96.2%) patients and only 2 (3.8%) patients had universal vitiligo.

Ophthalmologic examination was done in all patients. Eighteen patients (34.6%) had periocular depigmentation. Poliosis was seen in 3 (5.7%) patients [Figure 1]. Details of vitiligo patients with ocular findings are given in Table 1. Hypopigmented trabecular meshwork was seen in 5 (9.6%) patients, and one patient of these 5 patients had hypopigmented ciliary body also. Four of these 5 patients had periocular depigmentation. Statistically, significant association was found between periocular depigmentation and hypopigmented trabecular meshwork (P value 0.018). [Table 2 and Figure 2a, b] One patient had retinitis pigmentosa and periocular depigmentation. Relation between age, age at the onset, gender, periorbital depigmentation, and clinical type with ocular involvement is shown in Table 3. Most cases of ocular findings were detected in age group 1–20 years (26.3%). Ocular findings were present in 1 (6.7%) out of 14 patients having vitiligo for less than 5 years and 5 out of 32 (13.5%) having vitiligo for more than 5 years (P value 0.659). Statistically, significant association was found between periorbital depigmentation, (P value 0.005) and facial vitiligo (P value 0.019) with ocular findings.

Figure 1

Periorbital vitiligo with poliosis of eyebrow

Table 1

Details of vitiligo patients with ocular findings

Case no	Age (years)/sex	Duration of vitiligo	Clinical type of vitiligo	Ocular findings
1.	13/Female	1 month	Facial	Hypopigmented trabecular meshwork
2.	23/Female	6 years	Acrofacial	Hypopigmented trabecular meshwork
3.	26/Male	5 months	Facial	Retinitis pigmentosa
4.	13/Male	2 years	Facial	Hypopigmented trabecular meshwork and ciliary body band, poliosis of eye brows and eyelashes
5.	7/Male	1 year	Acrofacial	Hypopigmented trabecular meshwork, poliosis of eyelashes
6.	20/Male	4 years	Segmental (Face)	Hypopigmented trabecular meshwork

Table 2

Association of periocular depigmentation with hypopigmented trabecular meshwork

Periocular depigmentation	Number of cases with normal trabecular meshwork	Number of cases with hypopigmented trabecular meshwork
Present	13	4
Absent	34	1

P=0.018

Figure 2

(a) Periorbital vitiligo. (b) Gonioscopy of same patient showing hypopigmented trabecular meshwork (black arrow)

Table 3

Ocular findings in relation to age, age of onset, sex, periorbital depigmentation, and clinical type

	Number of cases with ocular findings (%)	Number of cases without ocular findings (%)	P value
Age group			P=0.38
1-20 years	5 (26.3)	14 (73.7%)
21-40 years	1 (4.5)	21 (91.5)
41-60 years	0 (0.0)	11 (100)
Age of onset			P=0.598
1-10 years	2 (15.4)	11 (84.6)
11-20 years	3 (16.7)	15 (83.3)
21-30 years	1 (16.7)	5 (83.3)
31-40 years	0 (0.0)	7 (100)
>40 years	0 (0.0)	8 (100)
Sex			P=0.503
Male	4 (14.3)	24 (85.7)
Female	2 (8.3)	22 (91.7)
Periorbital depigmentation			P=0.005
Present	5 (29.4)	12 (70.6)
Absent	1 (2.9)	34 (97.1)
Clinical type
Vitiligo vulgaris	19 (100)	0 (0)	P=0.019
Acrofacial	8 (80)	2 (20)
Facial	6 (66.7)	3 (33.3)
Acral	7 (100)	0 (0)
Focal	3 (100)	0 (0)
Mucosal	1 (100)	0 (0)
Vitiligo universalis	2 (100)	0 (0)
Segmental	0 (0)	1 (100)

In addition to the skin, melanocytes are found in the retinal pigment epithelium (RPE) and the uveal tract. Melanocytes present in the choroid are responsible for constitutive eye pigmentation and protection against ultraviolet radiation. These cells are important for the degradation of toxic factors.[8] The retinal pigment epithelium is formed by a distinct type of melanocytes as the outermost layer of the retina. These melanocytes are involved in the metabolism of retinoids and rod outer segments and play a major role in vision. In vitiligo, mechanisms responsible for melanocyte destruction in the skin can affect melanocytes within iris and retina of the eyes as well.

There is well-known association of vitiligo with ocular disturbances in diseases such as Vogt-Koyanagi-Harada syndrome and Alezzandrini's syndrome. In Vogt-Koyanagi-Harada syndrome, vitiligo, poliosis, uveitis, alopecia, neurological, and auditory findings (meningismus and tinnitus) are seen. In Alezzandrini's syndrome, unilateral facial vitiligo, white hair, poliosis, unilateral retinal degeneration, and deafness are seen.[9] Depigmentation of the eyelids and poliosis of the eyebrows and eyelashes are commonly seen in vitiligo.[10] Uveitis has been reported in 4.8–19% of patients with vitiligo.[15711] Various fundal pigment disturbances described in vitiligo includes pigment clumps, focal hypopigmented spots, diffuse hypopigmentation, diffuse, focal, or sectoral atrophy of the RPE, or chorioretinal scars, choroidal nevi.[123] Ring-like peripapillary atrophy around the optic nerve was also observed.[610] There are some isolated reports of vitiligo occurring with tapetoretinal degeneration.[1213] Hypopigmented spots on the iris and angle, iris atrophy, heterochromic iris and pigmentation in anterior chamber were also noted.[51415] However, we could not find any of the above fundus/iris findings in our study except one patient with retinitis pigmentosa. We found a statistically significant finding of hypopigmented trabecular meshwork in periorbital vitiligo. Retinitis pigmentosa and retinitis pigmentosa-like syndromes are seen sporadically in patients with vitiligo, but the relationship between vitiligo and retinitis pigmentosa is difficult to assess.[12] Albert et al. postulated that on rare occasions there may be sufficient RPE degeneration to produce a retinitis pigmentosa-like syndrome in vitiligo.[2] In the present study, one patient with periorbital vitiligo had retinitis pigmentosa. Vitiligo patients can be more prone to dry eye syndrome as well.[7] However, in the present study, we have not done Schirmer's test to evaluate dry eye. As few studies showed a risk of dry eye in vitiligo, Schirmer's test may also be included in ocular examination in vitiligo.

The connection between the anatomic localization of vitiligo and ocular findings was also studied by various authors. Rosenbaum et al. reported an association between bilateral changes in the RPE with periorbital vitiligo and seizures.[16] Wagoner et al. suggested that periocular skin depigmentation is a frequent abnormality in patients with ocular findings.[11] Baskan et al. reported that ocular findings are primarily associated with periorbital and, to a lesser extent, genital vitiligo.[10] Pai et al. found that iris and fundus changes were maximum in cases with lesions located on the face, and angle changes were more significant in cases with genital vitiligo.[14] In the present study, a significant association (P value 0.005) between periorbital depigmentation and ocular findings was found.

In the present study, except one retinitis pigmentosa patient, none of the other patients complained of visual impairment. Retinitis pigmentosa patient had decreased visual acuity, and other patients had normal visual acuity. This is in corroboration with the study of Nordunt et al. and Pai et al.[1417] The lack of symptoms can be explained by the location of the destructive lesions, which lie in the periphery and not near the macula.[14] We could not find significant association between ocular findings in vitiligo with age, gender, age at the onset of disease, and disease duration; these results are in agreement with the findings of Baskan et al. and Pai et al.[1014]

Although the clinical relevance of these ocular findings is unclear, there are some medicolegal implications as ocular changes can develop with steroids and phototherapy. Doria et al. studied ocular changes in vitiligo patients treated with oral Photochemotherapy with psoralen using natural sunlight (PUVASOL).[18] Discrete dusty, golden, yellow deposits were noticed in cornea and lens after 6 months of therapy. Reversible visual acuity changes were seen. Fundus showed dull macular degeneration in patients above 30 years of age. Shah recommended that for medicolegal implications, baseline documentation prior to starting phototherapy or applying topical steroids around the eyes is important, especially in a patient with periorbital vitiligo.[19]

To conclude, there is a significant association of facial vitiligo and periorbital vitiligo with ocular findings. Hence, ocular examination should be a part of routine workup, especially if vitiligo lesions involve head and neck region. For medicolegal implications also, before starting phototherapy or applying topical steroids around eyes, baseline documentation of eye examination is important, especially in periorbital vitiligo cases. Future prospective studies, evaluating the progression of ophthalmic findings and its correlation with vitiligo need to be done.

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Conflicts of interest

There are no conflicts of interest.