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Summary Clinical Presentation Generalized, segmental/localized, universal, or acrofacial in distribution Amelanotic macules and patches Occasional repigmentation in vitiliginous skin Histologic Features Absence of melanin in keratinocytes No melanocytes detected by light microscopy and immunohistochemistry Absent to scant melanin in subjacent dermis/submucosa Vacuolar interface alteration, but no significant inflammatory infiltrate Differential Diagnosis Idiopathic guttate melanosis Tinea versicolor Post-inflammatory hypopigmentation Halo nevus Regressing melanoma Lichen sclerosus Takeaway Essentials Clinical Relevant Pearls Ideal biopsy is from the edge to allow for comparison of normal and vitiliginous skin compared with adjacent normal skin Pathology Interpretation Pearls No melanocytes detected by light microscopy and immunohistochemistry Immunohistochemical/Molecular Findings Fontana-Masson or Warthin-Starry for melanin and immunohistochemical studies with various melanocytic markers such as S-100 protein, Mart-1, MiTF-1, and HMB-45 show absence of melanin and melanocytes in well-developed lesion Melanocytic Nevi Common Acquired Melanocytic Nevus Clinical Features Vulvar nevi are relatively uncommon, occurring in up to 2.3 % of women presenting with pigmented vulvar lesions [35]