Vitiligo

Vitiligo, white spot disease, piebald skin, leukoderma.

Chronic, acquired autoimmune disease of the skin with depigmented, sharply defined macules due to loss of epidermal melanocytes.

• Prevalence: 0.5 - 2% worldwide
• Onset usually between the ages of 10 and 30
• Women = Men
• Frequently associated autoimmune diseases (e.g. Hashimoto's thyroiditis, type 1 diabetes mellitus)
• Positive family history in approx. 20 %

• Non-segmental vitiligo (NSV): Most common form, symmetrically distributed, often progressive.
  • Universal (>80 %), acrofacial, mucosal, focal.

• Segmental vitiligo (SV): Unilateral, dermatomal course, often early onset, stabilizes early.
   

Multifactorial:

• Genetic predisposition (>50 known susceptibility genes, e.g. HLA, TYR, NLRP1)
• Autoimmune reaction (CD8+ T cells, IFN-γ, CXCL10)
• Trigger factors: psychological stress, sunburn, trauma (Koebner phenomenon)
• Oxidative stress, neurogenic factors discussed.

• White, sharply defined, asymptomatic macules
• Hair in lesions often white (leukotrichia)
• Predilection sites: Face (perioral, periorbital), hands, feet, axillae, genital, periumbilical
• Koebner phenomenon: new spots after skin injuries.

• Clinical visual diagnosis, if necessary, Wood lamp (bright white fluorescent)
• Biopsy usually unnecessary (in case of doubt: absence of melanocytes)
• Laboratory screening for associated autoimmune diseases (e.g. thyroid).

Predilection sites: Face, hands, feet, armpits, genital area, periumbilical.
Segmental vitiligo arranged strictly unilaterally and dermatomerically.

• Gradual onset, slow progression
• Trigger: stress, sunburn, trauma
• No significant skin complaints, high psychosocial distress.

• Loss of epidermal melanocytes
• Early stage: lymphocytic infiltrate, interface dermatitis
• Melanophages in the dermis possible.

• High psychosocial stress
• Risk of sunburn in depigmented areas
• More frequent autoimmune diseases as concomitant diseases.

• Chronic course with progressive phases and relapses
• Segmental vitiligo often stable after brief progression
• Spontaneous repigmentation rare, therapy can have a favorable influence on the course
• Life expectancy unaffected.

No specific prevention; avoidance of sunburns and skin trauma may be helpful. Early diagnosis and treatment recommended.

1. Bergqvist C, Ezzedine K. Vitiligo: a review. Dermatology. 2020;236(6):571–592.
2. Seneschal J, Speeckaert R, Taieb A, et al. Worldwide expert recommendations for the diagnosis and management of vitiligo – Part 2: Treatment recommendations. J Eur Acad Dermatol Venereol. 2023;37(11):2185–2195.
3. Eleftheriadou V, Atkar R, Batchelor J, et al. British Association of Dermatologists guidelines for the management of vitiligo 2021. Br J Dermatol. 2022;186(1):18–29.
4. Perez-Bootello J, Cova-Martin R, Naharro-Rodriguez J, Segurado-Miravalles G. Vitiligo: pathogenesis and new and emerging treatments. Int J Mol Sci. 2023;24(24):17306.
5. Iwanowski T, Kolkowski K, Nowicki RJ, Sokolowska-Wojdylo M. Etiopathogenesis and emerging methods for treatment of vitiligo. Int J Mol Sci. 2023;24(11):9749.
6. Rosmarin D, Passeron T, Pandya AG, et al. Two phase 3 trials of ruxolitinib cream for vitiligo. N Engl J Med. 2022;387(16):1445–1455.
7. Ju HJ, Bae JM, Lee RW, et al. Surgical interventions for patients with vitiligo: a systematic review and meta-analysis. JAMA Dermatol. 2021;157(3):307–316.
8. Huang F, Hu D, Fan H, et al. Efficacy and safety of Janus kinase inhibitors in patients with vitiligo: a systematic review and meta-analysis of randomized controlled trials. Clin Pharmacol Ther. 2025;117(3):659–669.
9. Diotallevi F, Gioacchini H, De Simoni E, et al. Vitiligo, from pathogenesis to therapeutic advances: state of the art. Int J Mol Sci. 2023;24(5):4910.
10. Marchioro HZ, Silva de Castro CC, Fava VM, et al. Update on the pathogenesis of vitiligo. An Bras Dermatol. 2022;97(4):478–490.