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Original Investigation | Clinical Sciences

Cutaneous Vitiligo Following Management of Uveal Melanoma in 6 Patients

Pukhraj Rishi, MD1,2; Carol L. Shields, MD1; Kaitlin Patrick, BS1; Jerry A. Shields, MD1
[+] Author Affiliations
1Ocular Oncology Service, Wills Eye Institute, Thomas Jefferson University, Philadelphia, Pennsylvania
2On sabbatical from Shri Bhagwan Mahavir Vitreoretinal Services, Sankara Nethralya, Chennai, India
JAMA Ophthalmol. 2013;131(9):1174-1178. doi:10.1001/jamaophthalmol.2013.4254.
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Published online

Importance  The relationship of vitiligo to cutaneous melanoma is believed to be due to an immune response generated to melanoma antigens that cross-react with normal skin. There is little in the literature on the relationship between cutaneous vitiligo and uveal melanoma.

Objective  To describe the clinical profile, treatment, and outcome in patients with uveal melanoma who subsequently developed cutaneous vitiligo.

Design, Setting, and Participants  Retrospective case series of 6 patients with uveal melanoma who had developed cutaneous vitiligo and were examined at a tertiary eye care institution.

Main Outcome and Measure  Development of cutaneous vitiligo.

Results  The mean age at presentation was 62 years (range, 39-85 years). No patient had a personal history of cutaneous melanoma, autoimmune disease, or cutaneous vitiligo. The mean tumor basal diameter was 12.9 mm (median, 12.7 mm; range, 7-19 mm), with a mean thickness of 9.5 mm (median, 8.4 mm; range, 3-19 mm). Treatment included plaque radiotherapy in 4 patients and enucleation in 1 patient; 1 patient refused therapy. No patient had local tumor recurrence at the 71-month mean follow-up, but of the 3 patients who developed metastases at the 52-month mean follow-up, 2 were treated with a melanoma vaccine. The mean interval from initial presentation to onset of vitiligo was 77 months (range, 5-168 months). The vitiligo developed bilaterally with multiple well-defined lesions, affecting 5% to 40% of the cutaneous surface, generally in the upper body. During the 71-month mean follow-up (range, 4-205 months), there was 1 death.

Conclusions and Relevance  Patients with uveal melanoma can develop vitiligo spontaneously or following vaccine therapy. Involvement is multiple and bilateral, predominantly affecting the upper body.

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Figure 1.
Vitiligo in a Patient Treated With Plaque Radiotherapy

A 39-year-old man (patient 5) with an amelanotic melanoma (A) 9.9 mm in thickness on ultrasonography (B) was treated with plaque radiotherapy. Seventeen years later, the right eye displayed cataract (C) and regressed melanoma (D). Cutaneous vitiligo was found on both arms (E and F), and there was no evidence of metastatic disease.

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Figure 2.
Vitiligo in a Patient Treated With Enucleation and Immunotherapy

A 65-year-old woman (patient 2) with eyelid edema, chemosis, and total hyphema (A) of the left eye had an ill-defined large intraocular mass filling the globe on ultrasonography (B), necessitating enucleation. At the 28-month follow-up, with a prosthesis in place (C), systemic metastases were detected and immunotherapy with vaccination was provided (D). Fifty-nine months later, cutaneous vitiligo of the periocular region (E) and arms (F) was detected.

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