ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyDrug Reaction DataReferencesInformation for PatientsView all Images (132)
Other Resources UpToDate PubMed

Vitiligo in Adult

See also in: External and Internal Eye,Anogenital
Contributors: Sarah N. Robinson MD, Vivian Wong MD, PhD, Oyetewa Oyerinde MD, Callyn Iwuala BA, 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. Vitiligo is usually asymptomatic, and lesions can range in size from millimeters to centimeters. While any part of the body can be affected, vitiligo often demonstrates distinct patterns including symmetric involvement of the face, upper chest, hands, ankles, axillae, groin, and around orifices (eyes, nose, mouth, urethra, and anus), often favoring sites of frequent friction or trauma. Distribution can be either localized, as in segmental vitiligo, generalized as in vitiligo vulgaris, or universal. Generalized forms include vulgaris (widely distributed, scattered macules and patches) and acrofacial (distal extremities and face). Mucous membrane involvement is not uncommon in generalized cases. In the universal form, nearly 100% of the body surface area is depigmented.

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.

Vitiligo occurs in equal proportions regardless of age, sex, or ethnicity. 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, two leading hypotheses include the following: 1) a host attack on normal melanocytes; and 2) intrinsic melanocyte defects. Genetic predisposition and trauma are other risk factors for vitiligo development. Exposure to depigmenting agents causes a vitiligo-like leukoderma in susceptible individuals. While the majority of 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 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).

Codes

ICD10CM:
L80 – Vitiligo

SNOMEDCT:
56727007 – Vitiligo

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

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, anti-centromere, anti-Scl-70 antibody. Associated with Raynaud phenomenon, arthralgias, mat telangiectasias, and CREST syndrome.
  • Albinism, piebaldism, and other genetic disorders – Begin in infancy.
  • Lichen sclerosus – Look for sclerotic plaques, often in the genital area; can be severely pruritic.
  • Medication-induced or chemical-induced leukoderma – Look for history of certain medications or chemicals.
  • Onchocerciasis – Shins are common site of involvement. Suspect if patient is coming from endemic area (Africa, Central or South America).
Hypopigmented macules:
  • Tinea versicolor – Potassium hydroxie (KOH) positive. Mild scale noted; often seen in the shoulders, 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 post-inflammatory hypopigmentation.
  • Leprosy – Lesions are usually hypopigmented, not depigmented. Some can have an erythematous border. Lesions are anesthetic. 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 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

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

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.

Subscription Required

References

Subscription Required

Last Reviewed:07/26/2017
Last Updated:02/07/2021
Copyright © 2021 VisualDx®. All rights reserved.
Vitiligo in Adult
See also in: External and Internal Eye,Anogenital
Vitiligo : Dorsum of hand, Face, Fingers, Hair color change, Symmetric extremities distribution, Depigmented macules/patches
Clinical image of Vitiligo
A white macule and a similar patch on the forearm.
Copyright © 2021 VisualDx®. All rights reserved.