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Vitiligo

Essentials

  • Vitiligo is an autoimmune disease characterised by slowly progressing patches of depigmented skin throughout the body.
  • Other autoimmune diseases occur in 10-15% of the patients. The most common are thyroid autoimmune diseases.
  • Treatment is most successful if it is started at an early stage of the disease.
  • Vitiligo patches tend to sunburn easily.

Epidemiology and pathophysiology

  • Vitiligo affects 0.5-2% of the population. Vitiligo is encountered in all races and equally between men and women.
  • Almost half of patients are under 20 years of age at disease onset.
  • According to different studies, 10-50% of patients also have close relatives with vitiligo or other autoimmune diseases.
  • The mode of inheritance for vitiligo is not known. Multiple susceptibility loci have been identified. Regulation disturbances affecting multiple genes are probable as is incomplete penetrance.
  • Vitiligo is an autoimmune disease in which cytotoxic CD8-positive T cells destroy epidermal melanocytes. The underlying precipitating factor is unknown.
  • Skin damage or sunburn may trigger vitiligo (Köbner phenomenon). Many patients connect the onset of vitiligo with pregnancy or mental stress.

Symptoms

  • Vitiligo patches (pictures 1 2 3) usually occur symmetrically in the limbs (pictures 4 5 6).
  • The typical sites on the trunk are the umbilical area and around the nipples. In the face depigmentation characteristically occurs around the mouth and eyes.
  • Light-coloured patches may also occur in the scalp hair or in hair on other areas of the body.
  • The patches usually progress gradually, and spontaneous repigmentation occurs only rarely.
  • Segmental vitiligo, which is unilateral linear or blotch-like, occurs more rarely. It develops quickly within 3-4 months and then ceases to progress.
  • Affected skin shows an almost complete absence of melanocytes.
  • The depigmented areas are prone to sunburn and must be protected with clothing or sunscreen with a SPF of at least 30.

Investigations

  • An association may exist between vitiligo and other autoimmune disorders.
  • Laboratory tests in vitiligo
    • Thyroid disease screening Examining a Patient with a Thyroid Complaint (thyroid abnormalities are present in about 20% of cases, but the symptom onset does not usually coincide with that of vitiligo)
      • TSH, free T4
      • TPO antibodies and antithyroglobulin antibodies. These identify patients who have an increased risk of developing autoimmune thyroiditis.
    • As required: basic blood count with platelets, antinuclear antibodies, fasting blood glucose.

Differential diagnosis

  • Diagnosis is made on the basis of clinical findings. If necessary, histopathological testing may be used to confirm diagnosis.
  • The skin lesions in pityriasis versicolor Pityriasis Versicolor are pale in the summer and may be confused with vitiligo. Pityriasis versicolor lesions are associated with slight scaling which is not a feature in vitiligo.
  • Lichen sclerosus of the vulva or penis Lichen Sclerosus may resemble vitiligo. A biopsy will assist in diagnosis.

Treatment

  • No definitely effective treatment is available.
  • In segmental vitiligo the treatment result is especially poor.
  • It may be decided, in agreement with the patient, not to attempt to treat vitiligo.
  • In light skinned patients, the patches are hardly noticeable during the winter months.
  • Many patients are content if the most visible lesions can be camouflaged with specially chosen make-up or self-tanning cosmetic products.
  • The patient must protect his/her skin against the sun as vitiligo patches are prone to sunburn, and if unaffected skin areas become suntanned the patchiness of the skin will be emphasised.
  • The possible treatment of vitiligo is the responsibility of a dermatologist.
    • Potent glucocorticoid creams may be used for vitiligo that is limited to a small area on the trunk or limbs, once daily for a maximum of 3 months or in 2-week periods with 2-week pauses in between for a maximum of 6 months. Patients should be monitored for potential dermatological adverse effects of glucocorticoids.
    • Tacrolimus is the first-line topical therapy in facial vitiligo. Long-term treatment and maintenance treatment are possible.
    • Narrowband UVB phototherapy may be used when vitiligo covers > 5-10% of the surface area of the skin.
    • The treatment response is best in young patients with vitiligo that has appeared recently. The response is usually worst on limbs.
    • New therapies, such as Janus kinase (JAK) inhibitors, are under development.

Quality of life

  • Vitiligo impairs the patient's quality of life to the same degree as psoriasis, atopic dermatitis or hand dermatitis.
  • In some cultures vitiligo is considered particularly traumatic. A historical/cultural explanation can probably be found in the differential diagnosis: leprosy and late stage syphilis are also associated with white patches.
  • A specialized care unit can be consulted regarding treatment options for vitiligo, if needed.
  • At least in specialist care, the management protocol should include the use of a quality of life questionnaire specific to the patient's condition (DLQI = Dermatology Life Quality Index http://www.dermatology.org.uk/downloads/dlqifinnish.pdf).

    References

    • Taieb A, Alomar A, Böhm M ym. Guidelines for the management of vitiligo: the European Dermatology Forum consensus. Br J Dermatol 2013;168(1):5-19. [PubMed]
    • Frisoli ML, Harris JE. Vitiligo: Mechanistic insights lead to novel treatments. J Allergy Clin Immunol 2017;140(3):654-662. [PubMed]
    • Rodrigues M, Ezzedine K, Hamzavi I ym. New discoveries in the pathogenesis and classification of vitiligo. J Am Acad Dermatol 2017;77(1):1-13. [PubMed]
    • Rodrigues M, Ezzedine K, Hamzavi I ym. Current and emerging treatments for vitiligo. J Am Acad Dermatol 2017;77(1):17-29. [PubMed]
    • Dahir AM, Thomsen SF. Comorbidities in vitiligo: comprehensive review. Int J Dermatol 2018;57(10):1157-1164. [PubMed]