Last Updated: 2023-07-07

Author(s): Anzengruber F., Navarini A.

ICD11: ED63.0

White spot disease

Acquired disease in which CD8+ T-lymphocytes attack melanocytes leading to depigmentation of the skin.

  • Prevalence: approx. 1% (depending on source: 0.1%-4%)
    • Ethnic differences:
      • India: approx. 2-4%
      • Generally, patients with black skin colour are more often affected
  • Peak age: 10-30 years, but any age is affected
    • 50% of cases before 20 yrs.
    • 25% of cases before the age of 10
  • Women = men
  • Familial clustering in 30-40%

  • Generalised vitiligo (90%)
    • Acrofacial: face/ distal extremities
    • Vulgaris: irregular
  • Localised vitiligo
    • 2% of cases
    • Focal: not located within a dermatome
    • Segmental: Arranged within a dermatome
    • Vitiligo of the mucosa: 0.5% of pat.
    Universal vitiligo
    • 8% of cases
    • > 80% of body surface area

As yet not explained in detail.

  • Possible aetiologies:
    • Autoimmunological
    • Neural pathologies (segmentally arranged depigmentation)
    • Self-destruction hypothesis
      • During melanin synthesis, self-destruction of melanocytes occurs due to a lack of protective mechanisms.
    • Growth factor deficiency
      • Melanocytes are not destroyed according to this theory, but are not formed due to growth factor deficiency
    • Genetic factors
      • Susceptibility loci: AIS 1-3, NALP1
  • Possible triggers:
    • Sunburn
    • Psychological stress
    • Thyreotoxicosis
    • Mechanical trauma
    • Birth
  • Association with other conditions: 
    • Thyroid disorders
      • Hyper- and hypothyroidism
        • Morbus Graves
        • Hashimoto's thyroiditis
    • Endocrinopathies
      • Pernicious anaemia (1-8%)
      • Diabetes mellitus type I and II
      • Morbus Addison
    • Autoimmune diseases such as
      • Alopecia areata
      • Lupus erythematosus
      • Myasthenia gravis
      • Pemphigus vulgaris
      • Morbus Crohn's
      • Systemic scleroderma
      • Biliary cirrhosis
    • Neoplasia
      • Malignant melanoma, in the context of therapy with:
        • Interferon, interleukin or vaccine therapies
        • Immune checkpoint therapies e.g. ipilimumab

Sharply demarcated, often symmetrical, partly confluent, pigment-free, whitish macules. In some cases an erythematous border is visible (dermatitis circinata Miescher). Hair arising from follicles in the affected areas typically turn whitish (poliosis circumscripta).

  • Clinic
    • Sufficient for diagnosis in most cases
    • Examination with the Wood light
  • If necessary, biopsy
  • Lab
    • BB, TSH, fT3, fT4, thyroid antibodies, APCA, ANAs, daily blood glucose profile
  • In case of scaling
    • Tape strips / KOH preparation e.g. of pityriasis versicolor alba
  • Ophthalmological consultation e.g. to seek asymptomatic uveitis or retinal pigmentary defect


Mostly on the face, hands, axillae / nipples / periumbilical or genitoanal region. First depigmentation is often seen periorally. Watch out for traces of tooth paste around the mouth in children - this imitates perioral vitiligo in the Wood light examination. Wiping with a wet tissue helps!

Decrease or absence of melanin and melanocytes (melanin and melan-A staining).

  • Eye disorders (uveitis)
  • Presbycusis

Frequent progression in relapses.

  1. Fain PR et al. (2003) A genomewide screen for generalized vitiligo: confirmation of AIS1 on chromosome 1p31 and evidence for additional susceptibility loci. Am J Hum Genet 72: 1560-1564
  2. Handa S et al. (2003) Epidemiology of childhood vitiligo: A study of 625 patients from North India. Pediatric Dermatol 20: 207-210
  3. Scherschun L (2001) Narrow-band ultraviolet B is a useful and well-tolerated treatment for vitiligo. J Am Acad Dermatol 44: 999-1003
  4. Taieb A et al. (2013) Vitiligo European Task Force (VETF); European Academy of Dermatology and Venereology (EADV); Union Européenne des Médecins Spécialistes (UEMS). Guidelines for the management of vitiligo: the European Dermatology Forum consensus. Br J Dermatol 168:5-19
  5. Travis LB et al. (2003) Successful treatment of vitiligo with 0.1% tacrolimus ointment. Arch Dermatol 139: 571-574
  6. Parsadet et al. (2003) Effectiveness of oral Gingko biloba in treating limites slowly spreading vitiligo. Clin Exp Dermatol 28: 285-287