Key Facts

Vitiligo is the most common depigmenting disorder. It is a chronic condition with significant psychological impact, particularly in individuals with darker skin. Autoimmune mechanisms are central to pathogenesis.

Epidemiology

• Prevalence: ~0.5–1% globally
• Equal sex distribution
• Onset: 50% before age 20; any age
• All ethnicities; more cosmetically apparent in Fitzpatrick types IV–VI
• Family history: ~15–20% (polygenic)

Aetiology

• Autoimmune: CD8+ cytotoxic T-lymphocytes target melanocytes — predominant theory
• Genetic: polygenic; associations with HLA-A2, NLRP1, CTLA4, PTPN22
• Oxidative stress: melanocyte susceptibility to oxidative damage (hydrogen peroxide accumulation)
• Neural hypothesis (segmental vitiligo): neuropeptide-mediated melanocyte damage
• Autoimmune associations: thyroid disease (~20%), pernicious anaemia, Addison's, type 1 DM, alopecia areata

Pathophysiology

• Autoimmune CD8+ T-cells → melanocyte apoptosis via IFN-gamma and CXCL10 → JAK-STAT pathway
• Complete absence of melanocytes in depigmented patches (confirmed by biopsy)
• Koebner phenomenon: trauma-induced depigmentation at injured sites

Non-Segmental Vitiligo (85%)

• Well-defined, chalky-white, depigmented macules/patches
• Bilateral and symmetrical
• Predilection for: periorbital, perioral, hands, feet, genitalia, axillae (areas of friction/trauma)
• Koebner phenomenon
• Progressive; may wax and wane
• Hair within patches may become white (leukotrichia)

Segmental Vitiligo (15%)

• Unilateral, quasi-dermatomal distribution
• Earlier onset (often childhood)
• Rapid onset then stabilises
• Less association with autoimmune disease
• Poorer response to medical therapy; better response to surgical (melanocyte transfer)

Variants

• Acrofacial: fingers, toes, face — common presentation
• Universal vitiligo: >80% body surface depigmented — rare
• Focal: isolated patch(es) not fitting other patterns

Red Flags

• Rapidly progressive vitiligo — consider systemic autoimmune disease
• Associated features: fatigue, weight change (thyroid), postural hypotension (Addison's)
• Poliosis (white eyelashes) + vitiligo + deafness + uveitis → Vogt-Koyanagi-Harada syndrome

Bedside

• Wood's lamp (365 nm UV): enhances contrast — depigmented patches fluoresce bright white; essential for detecting early/subtle lesions in fair-skinned patients
• Clinical photography: baseline documentation for monitoring

Bloods

• TFTs + anti-TPO antibodies: screen for thyroid autoimmune disease (all patients)
• FBC: pernicious anaemia screen
• Vitamin B12: pernicious anaemia
• Blood glucose / HbA1c: type 1 diabetes
• 9 am cortisol: if Addison's suspected (fatigue, hypotension, hyperpigmentation elsewhere)

Biopsy

• Not routinely required for typical vitiligo
• If diagnostic uncertainty: biopsy shows complete absence of melanocytes (Melan-A/HMB-45 negative)

Special Tests

• VIDA score (Vitiligo Disease Activity): assesses disease stability over previous 6 months
• Body surface area assessment: quantifies extent for treatment decisions

Non-Pharmacological

• Sun protection: SPF 50+; depigmented skin is very sensitive to UV; also prevents tanning contrast
• Camouflage: cosmetic camouflage (Covermark, Dermablend) — available on NHS
• Psychological support: significant psychosocial impact — CBT, counselling, support groups (Vitiligo Society)
• Patient education: chronic condition; treatment aims for repigmentation but not guaranteed cure

Pharmacological — Limited Vitiligo (<10% BSA)

• Potent topical corticosteroids: clobetasol propionate 0.05% or mometasone furoate 0.1% — OD for 2 months on, 2 weeks off; max 3–6 months
• Topical calcineurin inhibitors: tacrolimus 0.1% ointment or pimecrolimus 1% cream — preferred for face and flexures (steroid-sparing); BD for 6+ months
• Ruxolitinib 1.5% cream (JAK inhibitor): NICE approved — for non-segmental vitiligo; applied BD

Pharmacological — Widespread Vitiligo (>10% BSA)

• Narrowband UVB phototherapy: first-line — 2–3 sessions/week for 6–12 months; repigmentation begins from perifollicular melanocytes
• May be combined with topical corticosteroids or calcineurin inhibitors

Surgical (Stable Segmental Vitiligo)

• Autologous melanocyte transplantation: punch grafting or melanocyte-keratinocyte suspension transfer
• Requires disease stability for ≥12 months

Depigmentation (Universal Vitiligo)

• Monobenzyl ether of hydroquinone 20%: for patients with >50–80% depigmentation who choose to depigment remaining skin
• Irreversible — careful counselling required

Referral Criteria

• Dermatology: extensive disease, facial involvement, treatment failure, consideration for phototherapy/surgery
• Psychology: significant psychosocial impact
• Endocrinology: if associated autoimmune conditions identified

• Chronic, unpredictable course — spontaneous repigmentation in ~10–20%
• Treatment: ~50–75% achieve some repigmentation with phototherapy (6–12 months)
• Facial and trunk vitiligo respond best to treatment; acral areas (hands, feet) respond poorly
• Segmental vitiligo: stabilises early; good surgical candidate
• Non-segmental: progressive in most; relapses common after treatment
• No effect on physical health but significant psychological morbidity

Vitiligo Classification

Treatment Response by Site