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- An acquired depigmentation of the skin, which correlates with a loss of epidermal melanocytes. There are three clinical variants, each with subtypes.
- Localized: often in childhood, rapid onset then stabilizes. Involvement of hair is common early in the course; lacks associated autoimmune diseases
- Focal: few lesions, random distribution
- Segmental: Lesions occur within a dermatome (mostly trigeminal) or may follow Blaschko lines. Lesions usually stop abruptly at the midline.
- Mucosal: only mucosal surfaces involved
- Generalized/nonsegmental (most common variant): progressive, with flares, commonly associated with autoimmunity. Common locations are acral, periorificial, and in sites sensitive to pressure/friction (Koebner phenomenon).
- Vulgaris: most common subtype; scattered macules; often symmetric, wide distribution; mostly hands, axillae, and groin
- Acrofacial: on distal extremities and face
- Mixed: coexistence of above
- 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) scores.
- Other 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.
- System(s) affected: skin, mucous membranes
- Synonym(s): leukoderma
- 50% begin before age 20 years, peak in females: 1st decade; males: 5th decade. Onset earlier with positive family history; can appear as early as 6 weeks
- Predominance: male = female; however, females are more likely to seek treatment.
- No race or socioeconomic predilection
Etiology and Pathophysiology
Most likely a spectrum of disorders with a common phenotype and multiple mechanisms contribute to the pathology (convergence theory).
- Genetic: See “Genetics.”
- 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 lesional biopsies
- Oxidative stress from elevated H2O2 and NO and decreased catalase and erythrocyte glutathione
- Polygenic/multifactorial inheritance
- 20% of patients report affected relative, but monozygotic twins have only 23% concordance.
- HLA haplotypes, small nucleotide polymorphisms, and specific genes are all possible contributors.
- 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%
- Elevated antinuclear antibodies in up to 40%
- Elevated thyroperoxidase antibodies in 50%
- Less common
- Systemic lupus erythematosus
- Inflammatory bowel disease
- Melanoma (may be a sign of positive outcome of 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.
Associated with Hashimoto thyroiditis in a significant portion of children. Screening at onset and possibly annually may be beneficial.