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Vitiligo in Adult

See also in: External and Internal Eye,Anogenital
Contributors: Serena Dienes BSc, Oyetewa Oyerinde MD, Callyn Iwuala MD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Vitiligo is an acquired type of leukoderma characterized by well-circumscribed chalk-white depigmented macules or patches.

Two main clinical categories exist: nonsegmental vitiligo and segmental vitiligo.

Nonsegmental vitiligo can affect any part of the body, but it often involves the face, upper chest, hands, ankles, axillae, groin, and around orifices (eyes, nose, mouth, urethra, and anus), favoring sites of frequent friction or trauma. Distinct patterns include acrofacial involvement (distal extremities and face), focal (localized), generalized (widely distributed), and universal vitiligo (close to 100% of body surface area involved). Nonsegmental vitiligo may coexist with segmental vitiligo. Pure mucosal vitiligo may occur, and mucous membrane involvement is not uncommon in generalized cases.

Vitiligo occurs in equal proportions regardless of sex or race / ethnicity. There is a bimodal pattern of onset, first in childhood and then in adulthood. The adult-onset population accounts for approximately two-thirds of cases and has a mean onset of 34 years. The natural progression of the disease is unpredictable, ranging from insidious to rapid in onset. Years of stable, nonprogressive disease can be observed with the disease subsequently taking an unexpected rapid trajectory.

While the precise etiology of vitiligo remains debated, the leading hypothesis implicates both genetic and environmental factors that lead to an innate immune response resulting in CD8+ T-cell activation, release of interferon-γ (IFN-γ), and an associated inflammatory cascade in keratinocytes via the JAK/STAT pathway.

Environmental factors include mechanical trauma and/or exposure to depigmenting agents.

While most vitiligo patients are otherwise healthy, an association with autoimmune thyroid dysfunction (hyperthyroidism or hypothyroidism) has been demonstrated. In new-onset vitiligo patients with systemic symptoms, thyroid screening with antithyroid peroxidase (TPO) antibody and a serum thyrotropin is recommended. Additional associations include endocrinopathies, such as diabetes mellitus (Type 1, Type 2) and Addison disease, along with other autoimmune processes. Rarely, it may exist as part of polyglandular autoimmune syndrome, particularly a type III syndrome (eg, Hashimoto thyroiditis, vitiligo, or alopecia areata and/or another organ-specific autoimmune disease). Vitiligo may accompany halo nevi. New-onset vitiligo may be seen in patients with metastatic melanoma. It can occur spontaneously and may herald metastatic disease, or it can be triggered by immunotherapy such as with BRAF inhibitors or PD-1 inhibitors. In the latter setting, it is considered a good prognostic sign. Rarely, vitiligo may be associated with uveitis.

Related topic: segmental vitiligo

Codes

ICD10CM:
L80 – Vitiligo

SNOMEDCT:
56727007 – Vitiligo

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Depigmented macules:
  • Discoid lupus erythematosus – Presents with atrophy, telangiectasia, and follicular plugging, which are absent in vitiligo.
  • Scleroderma – Depigmented vitiligo-like macules or patches may occur in scleroderma; however, there is perifollicular retention of pigment, which is a differentiating clinical sign. Look for associated sclerotic skin. Check antinuclear antibodies (ANA) with nucleolar or speckled pattern, anticentromere, anti-Scl-70 antibody. Associated with Raynaud phenomenon, arthralgias, matted telangiectasias, and CREST syndrome.
  • Oculocutaneous albinism, Piebaldism, and other genetic disorders – Begin in infancy.
  • Lichen sclerosus – Look for sclerotic plaques, often in the genital area; can be severely pruritic.
  • Drug-induced hypopigmentation or Chemical leukoderma – Look for history of certain medications or chemicals.
  • Onchocerciasis – Shins are a common site of involvement. Suspect if the patient is coming from an endemic area (Africa, Central or South America).
Hypopigmented macules:
  • Tinea versicolor – Potassium hydroxide (KOH) positive. Mild scale noted; often seen on the shoulders and upper trunk.
  • Pityriasis alba – Typically affects the cheeks of atopic individuals; presents with hypopigmented, not depigmented, macules with ill-defined borders.
  • A history of prior trauma or skin inflammation can usually be elicited in cases of Postinflammatory hypopigmentation.
  • Leprosy – Lesions are usually hypopigmented, not depigmented. Some can have an erythematous border. Lesions are anesthetic. The patient must have recently lived in an endemic area.
  • Nevus depigmentosus – Common on the trunk; usually present since birth. Despite its name, it is hypopigmented.
  • Nevus anemicus – Not a true pigmentary disorder, but rather a localized area of relative vasoconstriction in the skin secondary to increased sympathetic supply.
  • Idiopathic guttate hypomelanosis – Characteristic pattern and shape of lesions is different from vitiligo, including well-demarcated, 0.4- to 0.7-mm macules that do not coalesce, are symmetric, and involve the extensor forearms and shins; the face is rarely involved.
  • Cutaneous T-cell lymphoma (mycosis fungoides) – Can have an associated scale. Lesions are usually hypopigmented, not depigmented.
  • Morphea – Look for sclerotic plaques.

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed:01/29/2024
Last Updated:02/08/2024
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Patient Information for Vitiligo in Adult
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Vitiligo in Adult
See also in: External and Internal Eye,Anogenital
A medical illustration showing key findings of Vitiligo : Face, Fingers, Depigmented macules/patches
Clinical image of Vitiligo - imageId=779676. Click to open in gallery.  caption: 'A white macule and a similar patch on the forearm.'
A white macule and a similar patch on the forearm.
Copyright © 2024 VisualDx®. All rights reserved.