Pukhraj Rishi1, Carol L Shields, Kaitlin Patrick, Jerry A Shields. 1. Ocular Oncology Service, Wills Eye Institute, Thomas Jefferson University, Philadelphia, Pennsylvania2On sabbatical from Shri Bhagwan Mahavir Vitreoretinal Services, Sankara Nethralya, Chennai, India.
Abstract
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.
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.