脈絡膜白斑：画像所見、臨床症状、および診断上の考慮事項に関する系統的レビュー

To synthesize and critically appraise the available evidence on choroidal vitiligo, integrating clinical presentation, multimodal imaging characteristics, and diagnostic differentiation from mimics, while proposing a set of diagnostic criteria based on literature synthesis and authors' expert consensus.

A systematic search of PubMed, Embase, and Cochrane Reviews through July 2025 identified English-language studies reporting on choroidal vitiligo. Two reviewers independently screened, extracted data, and assessed methodological quality for case reports and case series. Narrative synthesis and descriptive statistics were used to summarize the findings.

Eight studies met inclusion criteria, comprising 16 patients (28 eyes) from 5 countries. The majority of patients (75%) were asymptomatic, middle-aged females, with bilateral involvement in 88% of cases and preserved visual acuity (≥20/25 in 81% of eyes). Multimodal imaging consistently revealed well-demarcated choroidal hypopigmentation with intact retinal architecture and no intraocular inflammation. OCT demonstrated preserved retinal layers with occasional mild choroidal thinning. Fundus autofluorescence often showed scleral hyper-autofluorescence; FA/indocyanine green angiography showed mild window defects without leakage. Cutaneous vitiligo was present in 63% of patients, with no systemic autoimmune disease reported.

Choroidal vitiligo is a rare, benign, and often incidental finding, best recognized through multimodal imaging. This review proposes clinical diagnostic criteria for primary choroidal vitiligo and highlights its key imaging signature to aid differentiation from neoplastic and inflammatory mimics. We recommend baseline multimodal imaging and follow-up at 6-12 months to confirm stability. Standardized imaging criteria and prospective studies are needed to better define prevalence, natural history, and systemic associations. Given that most included studies were single-case reports, the overall level of evidence is low.