What is Vitiligo?

Vitiligo is a skin disorder characterized by selective
and progressive loss of melanocytes due to a chronic
autoimmune inflammatory response.1,2 Vitiligo lesions are
completely lacking in melanin, non-scaly, and have
distinct margins.1 Vitiligo can affect people of any skin tone,
ethnicity, gender, and age.1,2

Current research suggests that vitiligo is caused by
autoimmune responses in genetically susceptible individuals.
In people with vitiligo, certain environmental factors may induce
stress responses in the skin, triggering new lesions or expansion
of existing lesions.3 Triggers may include:

  • Sunburn3
  • Chemical Exposure3
  • Physical Skin Trauma4
  • Psychological Stress4-6

In most cases, the specific trigger is unknown.3

About 25% to 50% of people with vitiligo have a relative in
their extended family with vitiligo and about 6% have siblings
with vitiligo, suggesting a genetic component.7

Types of vitiligo

Vitiligo is classified as two major types: segmental vitiligo and nonsegmental vitiligo.
The type of vitiligo a person has may be a factor in the course of their disease.1

Image of a person with segmental vitiligo

Segmental Vitiligo (SV)17:

  • Appears as depigmented patches of skin usually on one side of the body, confined to a single dermatome with partial or complete involvement1
  • Early involvement of melanocytes in hair follicles (leukotrichia–white hair) and rapidly progressive with limited course2
  • Accounts for 5% to 16% of overall vitiligo cases1

Image of a person with nonsegmental vitiligo

Nonsegmental Vitiligo (NSV)17:

  • Appears as depigmented patches of skin of varying sizes, usually on both sides of the body, and tends to show up symmetrically2
  • Body hairs within lesions usually remain pigmented, although hair depigmentation may occur with disease progression2
  • Includes a subgroup called mixed vitiligo that has characteristics of both SV and NSV2
  • More common than segmental vitiligo1,2,8

How common is vitiligo?

Between 0.5% to 2.0% of the global population is estimated to have vitiligo.1

As of 2020, there are an estimated
1.9 to 2.8 million people with vitiligo in the US, but ≈40% of them are undiagnosed.8

Vitiligo affects people of all ethnic groups, skin types, and sexes.1,2

When do people develop vitiligo?1

≈25%

before the
age of 10

≈50%

before the
age of 20

≈70 to 80%

before the
age of 30

Diagnosis and Assessment

The diagnosis of vitiligo is usually based solely on physical examination and medical history, and does not require confirmatory laboratory tests, except to exclude other disorders. For patients with pale skin, diagnosis may be facilitated by the use of a Wood’s lamp, which is an ultraviolet irradiation device. Dermoscopy can be used to differentiate vitiligo from other depigmenting disorders and to assess disease severity, and disease progression.1

Vitiligo Measurement Scales

There are various methods for assessing and measuring the extent of vitiligo. In clinical trials, both subjective and objective measures are used.9 Several of the following measurement scales are commonly used in clinical trials.

Comorbidities and psychosocial impact

As an autoimmune condition, vitiligo can be associated with
thyroid disorders and other immune‑mediated diseases, such as1:

  • alopecia areata
  • rheumatoid arthritis
  • adult-onset diabetes
  • Addison’s disease
  • pernicious anemia
  • systemic lupus erythematosus
  • atopy
  • psoriasis

Vitiligo can be one of the most psychologically devastating
diseases in dermatology, causing isolation, impact on identity, loss
of self-esteem, and depression in some patients. Children and
adolescents with vitiligo may experience emotional distress,
self‑consciousness, stigmatization, and bullying.14

The impact on patients’ lives is often personal. Most patients
report some degree of lifetime emotional burden of vitiligo and
the emotional burden score is higher in patients with darker skin
types, who identify as females, and those with facial lesions.15
Those with genital lesions may also experience issues with
sexual intimacy.14

Prognosis

The evaluation of a patient with vitiligo requires a detailed history and skin examination to assess disease severity and individual prognostic factors.1

The following features have been described as markers of active disease or vitiligo progression4:

  • lesions appearing in response to trauma
  • trichrome lesions, which have a zone of hypopigmented skin near the border
  • inflammatory lesions
  • confetti-like lesions

Response to therapy often varies based on the location of the lesion, and is dependent on the density of hair follicles.16 The face, neck, trunk, and mid‑extremities respond best to therapy, while the lips and distal extremities are more resistant.1

Get To Know Jak In Vitiligo

Learn about the key role of the JAK-STAT pathway in vitiligo.

Explore The Science

References

1. Bergqvist C, Ezzedine K. Vitiligo: a review. Dermatol. 2020;236(6):571‑592. doi:10.1159/000506103

2. Ezzedine K, Lim HW, Suzuki T, et al. Revised classification/nomenclature of vitiligo and related issues: the Vitiligo Global Issues Consensus Conference. Pigment Cell Melanoma Res. 2012;25(3):E1-E13. doi:10.1111/j.1755-148X.2012.00997.x

3. Manga P, Elbuluk N, Orlow SJ. Recent advances in understanding vitiligo. F1000Res. 2016;5:F1000 Faculty Rev-2234. doi:10.12688/f1000research.8976.1

4. Rodrigues M, Ezzedine K, Hamzavi I, Pandya AG, Harris JE; Vitiligo Working Group. New discoveries in the pathogenesis and classification of vitiligo. J Am Acad Dermatol. 2017;77(1):1-13. doi:10.1016/j.jaad.2016.10.048

5. Henning SW, Jaishankar D, Barse LW, et al. The relationship between stress and vitiligo: evaluating perceived stress and electronic medical record data. PLoS One. 2020;15(1):e0227909. doi:10.1371/journal.pone.0227909

6. Silverberg JI, Silverberg NB. Vitiligo disease triggers: psychological stressors preceding the onset of disease. Cutis. 2015;95(5):255-262.

7. Genetic and Rare Diseases Information Center. Vitiligo. https://rarediseases.info.nih.gov/diseases/10751/vitiligo. Updated July 10, 2018. Accessed January 26, 2022.

8. Gandhi K, Ezzedine K, Anastassopoulos KP, et al. Prevalence of vitiligo among adults in the United States. JAMA Dermatol. 2021;158(1):43-50. doi:10.1001/jamadermatol.2021.4724

9. Dicle O. Assessment methods in vitiligo. J Pigment Disord. 2015;2(1):1‑2. doi:10.4172/2376-0427.1000160

10.  Rosmarin D, Pandya AG, Lebwohl M, et al. Ruxolitinib cream for treatment of vitiligo: a randomised, controlled, phase 2 trial. Lancet. 2020;396(suppl):110-120. doi:10.1016/S0140-6736(20)30609-7

11.   Hamzavi I, Jain H, McLean D, Shapiro J, Zeng H, et al. Parametric modeling of narrowband UV-B phototherapy for vitiligo using a novel quantitative tool: the vitiligo area scoring index. Arch Dermatol. 2004;140:677-683. doi: 10.1001/archderm.140.6.677

12.  Peralta-Pedrero ML, Morales-Sánchez MA, Jurado-Santa Cruz F, De la Torre-García ME, Cruz-Peralta ES, Olguín-García MG. Systematic review of clinimetric instruments to determine the severity of non‑segmental vitiligo. Australas J Dermatol. 2019;60(3):e178-e185. doi:10.1111/ajd.13008

13.   Batchelor JM, Tan W, Tour S, Yong A, Montgomery AA, Thomas KS. Validation of the vitiligo noticeability scale: a patient‑reported outcome measure of vitiligo treatment success. Br J Dermatol. 2016;174(2):386-394. doi:10.1111/bjd.14208

14.   Grimes PE, Miller MM. Vitiligo: patient stories, self-esteem, and the psychological burden of disease. Int J Womens Dermatol. 2018;4(1):32-37. doi:10.1016/j.ijwd.2017.11.005

15.  Narayan V, Uitentuis S, Luiten R, Bekkenk M, Wolkerstorfer A. Patients’ perspective on current treatments and demand for novel treatments in vitiligo. JEADV. 2021;35(3):744-748. doi:10.1111/jdv.16927

16.  Birlea SA, Goldstein NB, Norris DA. Repigmentation through melanocyte regeneration in vitiligo. Dermatol Clin. 2017;35(2):205-218. doi:10.1016/j.det.2016.11.015

17.  Faria AR, Tarlé RG, Dellatorre G, Mira MT, Castro CC. Vitiligo - part 2 - classification, histopathology and treatment. An Bras Dermatol. 2014;89(5):784-790. doi:10.1590/abd1806-4841.20142717