Vitiligo is an acquired depigmenting disorder characterized by destruction or functional loss of melanocytes, resulting in well-demarcated depigmented macules and patches. Autoimmunity, oxidative stress, genetic susceptibility, and neurogenic factors contribute to pathogenesis. Disease activity varies from stable to rapidly progressive. Management combines stabilization of active disease, repigmentation therapies (topical agents, phototherapy), surgical options for stable refractory lesions, and psychosocial support. Recent advances include topical JAK inhibitors and evolving systemic JAK strategies in select cases.
Epidemiology
- Prevalence ~0.5–2% worldwide; no strong sex predilection.
- Onset commonly before 30 years; family history in ~20–30%.
- Associations: other autoimmune diseases (thyroid disease, type 1 diabetes, pernicious anemia, alopecia areata), uveitis in rare subsets.
Pathophysiology
- Autoimmune T-cell–mediated melanocyte destruction; IFN-γ/CXCL9/10 axis central.
- Oxidative stress and intrinsic melanocyte fragility; Koebner phenomenon common.
- Genetics: polygenic susceptibility (NLRP1, PTPN22, HLA loci, TYR).
Clinical Features
- Non-segmental (generalized) vitiligo: bilateral, often acral/orificial; confetti-like lesions may indicate activity.
- Segmental vitiligo: unilateral dermatomal/segmental distribution; earlier onset, often rapidly stabilizes; more resistant to medical therapy but suitable for surgery when stable.
- Special sites: face (periorificial), hands/feet, bony prominences, axillae, genitalia; leukotrichia indicates poorer prognosis for repigmentation.
- Signs of activity: confetti depigmentation, trichrome lesions, Koebnerization, inflammatory borders.
Differential Diagnosis
- Pityriasis alba, tinea versicolor (KOH), postinflammatory hypopigmentation, lichen sclerosus, chemical leukoderma, nevus depigmentosus/nevus anemicus, hypomelanosis of Ito.
Diagnostic Workup
- Clinical diagnosis with Wood’s lamp accentuation.
- Screen for autoimmune thyroid disease (TSH ± TPO antibodies) based on risks/symptoms.
- No routine biopsy unless atypical; histology shows absence of melanocytes and lymphocytic interface changes in active edges.
Management
- Education and support
- Photoprotection reduces contrast and prevents sunburn on depigmented skin.
- Camouflage cosmetics, self-tanners; psychosocial support.
- Stabilization of active disease
- Short courses of oral corticosteroid “mini-pulse” (e.g., dexamethasone 2.5–5 mg on two consecutive days weekly for 6–12 weeks) in rapidly progressive cases under specialist care.
- Consider methotrexate or cyclosporine in select refractory rapidly progressive disease per protocols.
- Topical therapies
- Topical corticosteroids (mid-to-high potency for trunk/extremities; low potency for face/flexures) in limited areas for 8–12 weeks, then intermittently.
- Topical calcineurin inhibitors (tacrolimus 0.1% or pimecrolimus) especially for face/neck and intertriginous sites; good safety long-term.
- Topical JAK inhibitors: ruxolitinib 1.5% cream approved for nonsegmental vitiligo in many regions (ages ≥12); best for face; monitor for local AEs (acne, pruritus); systemic exposure is low.
- Phototherapy
- Narrowband UVB (NB-UVB) is first-line for generalized disease; 2–3 times weekly for months; best responses on face/neck; combination with topicals improves outcomes.
- Excimer laser (308 nm) for localized lesions.
- Counsel on realistic timelines (3–6 months for visible repigmentation; 12+ months for maximal effect).
- Surgical options (stable disease ≥6–12 months, usually segmental or focal)
- Suction blister epidermal grafting, split-thickness grafts, non-cultured epidermal cell suspensions, punch grafting; best for stable, refractory, cosmetically sensitive areas.
- Depigmentation therapy (extensive disease >50–60% with patient preference)
- Monobenzyl ether of hydroquinone 20% for gradual depigmentation of remaining pigmented skin; permanent; stringent photoprotection required.
- Special considerations
- Children: prioritize TCIs and NB-UVB; avoid prolonged potent steroids.
- Skin of color: address psychosocial impact and PIH risk from therapies/procedures; emphasize camouflage strategies.
Prognosis and Monitoring
- Course is unpredictable; many stabilize with treatment.
- Leukotrichia and acral involvement are poor prognostic markers.
- Relapses may occur; maintenance with intermittent topical therapy and periodic NB-UVB can help.
References (recent guidelines and key reviews)
- International/European vitiligo guidelines, 2022–2024.
- Trials of topical ruxolitinib and combination NB-UVB regimens, 2021–2024.
- Reviews on IFN-γ/CXCL10 pathway and systemic JAK strategies, 2022–2024.
- Surgical consensus statements for stable vitiligo, 2022–2024.