Vitiligo

White spot disease

Acquired chronic inflammatory autoimmune disease in which CD8⁺ tissue-resident T lymphocytes have a melanotoxic effect and lead to melanocyte loss and consecutive depigmentation via IFN-γ and CXCL10.

• Prevalence: approx. 0.5-2 % worldwide
• India: approx. 2-4 %
• Dark-skinned people more frequently affected
• Peak age: 10-30 years
• 50 % of cases before the age of 20
• 25 % before the age of 10
• Women = men
• Familial clustering: 30-40 %
   

• Localized vitiligo (~2 %)
  • Focal
  • Segmental
  • Mixed / not classifiable

• Generalized vitiligo (~90 %)
  • Acrofacial
  • Vitiligo vulgaris

• Universal vitiligo (~8 %)
• Mucosal vitiligo (~0.5 %)

• Autoimmunological (CD8⁺ T cells, IFN-γ, CXCL10)
• Oxidative stress
• Genetic susceptibility (including NLRP1, PTPN22, TYR, AIS1-3)
• Segmental form: neuronal theory
• Hypothesis of self-induced melanocyte toxicity

Possible triggers:
Sunburn, psychological stress, thyrotoxicosis, mechanical trauma, childbirth.

Associations:

• Autoimmune thyreopathies (Hashimoto's, Graves' disease)
• Pernicious anemia, diabetes mellitus type I and II, Addison's disease
• Alopecia areata, SLE, RA, psoriasis, Crohn's disease, PBC, scleroderma, myasthenia gravis
• Checkpoint inhibitor therapy (e.g. ipilimumab)

Sharply defined, often symmetrical, white macules. Often confluent. Frequently perioral, periorbital, on hands, axillae, genital area. Poliosis circumscripta typical. With active margins possibly reddish borders (Miescher's sign).

• Clinically confirmed
• Wood light: white-blue fluorescent
• Biopsy if unclear
• Laboratory diagnostics: BB, TSH, fT3, fT4, TPO-AK, TRAK, APCA, ANA, glucose profile
• Mycology for scaling (pityriasis versicolor)
• Ophthalmologic consultation for segmental vitiligo (exclusion of uveitis)
   

Face, perioral, hands, axillae, nipples, genitoanal, periumbilical. Caution with toothpaste residues in childhood.

Fluctuating course, often intermittent. Triggers can often be identified.

• Loss of melanocytes (Melan A staining negative)
• No epidermal atrophy
• Slight lymphocyte infiltrate may be present in active lesions
   

• Uveitis (rare, especially in segmental form)
• Hearing impairment (presbycusis)
• Psychological stress, social stigmatization
   

• Consistent UV protection (SPF ≥ 50)
• Avoid triggers (mechanical, emotional)
• Psychological support if required
   

Chronic, progressive and relapsing. Spontaneous repigmentation possible (perifollicular). Segmental form usually stable after 1-2 years. Partial remission with therapy frequent, complete repigmentation rare.

General:

• Sun protection (also with PUVA/UVB therapy)
• Treatment of associated diseases
• Psychosocial support
   

Topical therapy:

• Class III: Mometasone daily for 8 weeks, then 2×/week
• Class IV: Clobetasol
• Calcineurin inhibitors (tacrolimus 0.1 % / pimecrolimus 1 %)
• JAK inhibitor ruxolitinib 1.5 % cream (Opzelura®)
  • In CH since Feb. 2025 Swissmedic- and BAG-approved for non-segmental vitiligo ≤10 % KOF (mainly face)
• Camouflage, self-tanner (with SPF)
• Bleaching agent: hydroquinone/retinoid/dexamethasone combination (not offered at the USB)
   

Phototherapy:

• NB-UVB 311 nm: up to 60 % effectiveness
• PUVA (oral/topical)
• Excimer laser for focal infestation
• Combination with L-phenylalanine or Khellin (adjuvant)
   

Systemic therapy:

• Glucocorticoids (short-term, e.g. prednisone 1mg / kg body weight / day and tapered over 6 weeks)
• Beta-carotene (obsolete, cosmetic effect)
• Cyclosporin A (individual case, immunomodulatory)
• Off-label: oral JAK inhibitors (tofacitinib, ritlecitinib - not approved for vitiligo)
   

Surgical:

• Melanocyte-keratinocyte transplantation for stable segmental vitiligo (specialized centers)

1. Rosmarin D et al. Ruxolitinib cream for the treatment of vitiligo. N Engl J Med. 2022;387(2):144-153.
2. Swissmedic. Opzelura® approval for non-segmental vitiligo. February 2025.
3. Ezzedine K et al. Vitiligo: update and perspectives. Lancet. 2023;402(10398):389–402.
4. Taieb A et al. Guidelines for the management of vitiligo. Br J Dermatol. 2013;168:5-19.
5. Kuhn A et al. Pathogenesis and classification of vitiligo. J Eur Acad Dermatol Venereol. 2024;38(1):12-22.
6. Chen K et al. Role of tissue-resident memory T cells in vitiligo pathogenesis and treatment. J Invest Dermatol. 2023;143(5):1023-1032.
7. van Geel N et al. Position statement: emerging strategies for the treatment of vitiligo. J Eur Acad Dermatol Venereol. 2024;38(5):745-759.