• An acquired depigmentation of the skin, which correlates with a loss of epidermal melanocytes. There are two major clinical variants, each with subtypes.
  • Nonsegmental vitiligo: depigmented macules of varying size
    • Acrofacial: on distal extremities and face
    • Generalized: progressive, with flares, commonly associated with autoimmunity. Common locations are acral, periorificial, and in sites sensitive to pressure/friction (Koebner phenomenon).
    • Mixed: coexistence of nonsegmental and segmental
    • Mucosal (multifocal): only mucosal surfaces
    • Rare variants:
      • Ponctué: discrete, confetti-like macules
      • Inflammatory: peripheral erythematous rim
      • Trichrome: Tan zone is present between normal and depigmented skin.
      • Quadrichrome: as above but with marginal/perifollicular hyperpigmentation
      • Blue: Dermal melanophages give blue hue in areas affected by prior postinflammatory hyperpigmentation.
    • Universal: involves >80% of the body surface area (BSA). Most likely to have family history; comorbidities are common and associated with poorest quality-of-life (QOL).
  • Segmental vitiligo: Lesions occur within a dermatome (often trigeminal) or may follow Blaschko lines. Lesions usually stop abruptly at the midline; typically occurs earlier than nonsegmental vitiligo, is rapidly progressive, and involves melanocytes of hair follicles.
    • Bisegmental: bilaterally distributed
    • Plurisegmental: multiple segments
    • Unisegmental: single segment
  • Undetermined/unclassified:
    • Focal: few lesions, random distribution; may be able to later classify as one of the forms above after 1 to 2 years
    • Mucosal (focal type): only mucosal surfaces


  • 50% begin before age 20 years; females in 1st decade; males in 5th decade; onset earlier with positive family history; can appear as early as 6th weeks of life
  • Predominance: male = female; however, females are more likely to seek treatment.
  • No race or socioeconomic predilection

~1% in the United States and Europe (1); 0.1–8% in the world; highest in Gujarat, India at 8.8% (1)

Etiology and Pathophysiology

Most likely, a spectrum of disorders with a common phenotype and multiple mechanisms contribute to the pathology (convergence theory).

  • Autoimmune: humoral autoantibodies and skin-homing T cells
  • Neural: local or systemic dysregulation leading to excess neurotransmitters
  • Viral: direct melanocyte toxicity, cytomegalovirus (CMV), hepatitis C, and Epstein-Barr virus (EBV) found in lesion biopsies
  • Oxidative stress from elevated H2O2 and NO and decreased catalase and erythrocyte glutathione


  • Polygenic/multifactorial inheritance
  • 20% of patients report affected relative; monozygotic twins have only 23% concordance.
  • HLA haplotypes, small nucleotide polymorphisms, and specific genes are all possible contributors.

Risk Factors

  • Family history of vitiligo/autoimmune disorders
  • Personal history of associated conditions

Commonly Associated Conditions

  • Most common
    • Endocrine: thyroid disease (hypo-/hyperthyroidism), hypoparathyroidism, Addison disease, insulin-dependent diabetes
    • Dermatologic: psoriasis, atopic dermatitis, alopecia areata, chronic urticaria, halo nevi, ichthyosis
    • Pernicious anemia
    • Hypoacusis, rheumatoid arthritis
    • Ocular abnormalities in up to 40%
    • High antinuclear antibodies in up to 40%
    • Elevated thyroperoxidase antibodies in 50%
  • Less common
    • Systemic lupus erythematosus
    • Inflammatory bowel disease
    • Melanoma and other skin cancers
    • Syndromes: Alezzandrini; mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS); Schmidt; and autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED)
  • Age >50 years at onset should prompt investigation for associated conditions.

Pediatric Considerations
Associated with Hashimoto thyroiditis in a significant portion of children; screening at onset and possibly annually may be beneficial.

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