Vogt-Koyanagi-Harada syndrome associated with cutaneous malignant melanoma: an 11-year follow-up

To report a case of Vogt-Koyanagi-Harada (VKH) syndrome associated with cutaneous pigmented malignant melanoma (MM) and non-pigmented nodular metastasis after a 10-year recurrence-free interval.

Case report with long-term follow-up of 11 years. Ocular examinations included best-corrected visual acuity (ETDRS charts), fundus photography, fluorescein angiography, and computer-assisted perimetry. In addition, human leukocyte antigen (HLA) typing was performed.

A 48-year-old white female patient developed VKH disease 5 years after she had undergone surgical treatment of a superficial spreading melanoma on her back in 1991. The first symptoms were diffuse alopecia followed by growth of non-pigmented hair after 8 months. In our clinic, she presented 18 months later with cells and opacification in the vitreous, a macular and optic disc edema and atrophy of the retinal pigment epithelium (RPE). The anterior segment showed endothelial precipitates of the cornea, and a progressive secondary cataract. Fluorescein angiography detected a bilateral edema of the macula and the optic disc, and focal leakage in the RPE level. During follow-up the patient developed more cutaneous signs, such as vitiligo-like depigmentation and poliosis. A non-pigmented lymph node MM metastasis was diagnosed after a 10-year disease-free interval. HLA typing was positive for HLA-A*01, HLA-A*24, HLA-B*08, HLA-B*39, HLA-DRB1*03, and HLADRB1*11.

Our findings suggest that the described ocular findings of VKH disease may represent a component of a syndrome consisting also of melanoma-associated hypopigmentation. Within the framework of current concepts of immunity in patients with MM and VKH, the long recurrence-free interval might support the hypothesis of an autoimmune or hypersensitivity process against melanocytes. The use of immunosuppressive therapy in the treatment of VKH and its potential influence on the development of metastatic disease should be carefully reconsidered.