Vitiligo in Adult
See also in: External and Internal Eye,AnogenitalAlerts and Notices
Synopsis
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 race and 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).
- 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:06/08/2022
Last Updated:06/08/2022
Patient Information for Vitiligo in Adult
Overview
Vitiligo is a condition caused by the immune system turning against itself (an autoimmune disease). Immune cells of the body attack the color-producing (pigment-producing) cells in the skin, which leads to the formation of white (depigmented) patches that are often much larger than a thumbnail. In lighter skin colors, the difference between the lighter and darker skin can be difficult to see. In darker skin colors, the contrast between the normal pigmented skin and depigmented areas is greater than in lighter skin colors.Vitiligo may be seen with other autoimmune diseases, such as thyroid disease, alopecia areata, diabetes mellitus, Addison disease, and myasthenia gravis.
The way that vitiligo progresses varies greatly; it may remain in the area where it started (localized) or it may become more widespread.
Who’s At Risk
Vitiligo may start at any age but usually begins between the ages of 2 and 40. All races / ethnicities may be affected. Despite a common belief, vitiligo is not more common in Black individuals; however, the contrast between affected and normal skin is more obvious and potentially more cosmetically bothersome in individuals with darker skin colors.Signs & Symptoms
Vitiligo may affect areas prone to chronic minor injury, such as the fingers, knuckles, and elbows. It may also affect the face, upper chest, hands, underarms, and groin. It may be widespread, affecting both sides of the body (generalized); it may affect only one side of the body (segmental); or it may affect only one localized area (focal).Sharply defined white patches are seen. If the affected area contains hair, the hair may turn white.
Sometimes halo nevi can be seen, which is a mole surrounded by a circular white patch, resembling a halo.
Self-Care Guidelines
It is important to protect your skin from the sun, particularly the affected areas:- Avoid exposure to midday sun (10 AM to 3 PM).
- Wear a broad-spectrum, high sun protection factor (SPF higher than 30) sunscreen.
- Wear protective clothing and hats.
When to Seek Medical Care
It is not necessary to treat vitiligo, but seek medical evaluation if it becomes bothersome or if it is progressing.Treatments
Your doctor may try any of the following:- Topical steroid creams or ointments to help restore skin color. Use these with caution because of the risk of tissue damage (atrophy) with prolonged use, particularly on the face and skin fold areas.
- Topical nonsteroid medications, such as ruxolitinib (Opzelura) cream, tacrolimus (Protopic) ointment, and pimecrolimus (Elidel) cream.
- Ultraviolet (UV) light therapy or laser therapy.
- Superficial skin grafts, especially in hard-to-treat (recalcitrant) locations, such as over joints.
- For very large or widespread lesions, topical chemicals can remove color from (depigment) the normal skin.
Vitiligo in Adult
See also in: External and Internal Eye,Anogenital