Vitiligo

Etiology of vitiligo is unclear, but melanocytes are lacking in affected areas. Proposed mechanisms include autoimmune destruction of melanocytes, reduced survival of melanocytes, and primary melanocyte defects.

Vitiligo can be familial (autosomal dominant Autosomal Dominant Genetic disorders determined by a single gene (Mendelian disorders) are easiest to analyze and the most well understood. If expression of a trait requires only one copy of a gene (one allele)... read more with incomplete penetrance and variable expression) or acquired. Some patients have antibodies to melanin. Up to 30% have other autoimmune antibodies (to thyroglobulin, adrenal cells, and parietal cells) or clinical autoimmune endocrinopathies (Addison disease Addison Disease Addison disease is an insidious, usually progressive hypofunctioning of the adrenal cortex. It causes various symptoms, including hypotension and hyperpigmentation, and can lead to adrenal crisis... read more , diabetes mellitus Diabetes Mellitus (DM) Diabetes mellitus is impaired insulin secretion and variable degrees of peripheral insulin resistance leading to hyperglycemia. Early symptoms are related to hyperglycemia and include polydipsia... read more , pernicious anemia Autoimmune Metaplastic Atrophic Gastritis Autoimmune metaplastic atrophic gastritis is an inherited autoimmune disease that attacks parietal cells, resulting in hypochlorhydria and decreased production of intrinsic factor. Consequences... read more , and thyroid dysfunction). However, the relationship is unclear and may be coincidental. The strongest association is with hyperthyroidism Hyperthyroidism Hyperthyroidism is characterized by hypermetabolism and elevated serum levels of free thyroid hormones. Symptoms include palpitations, fatigue, weight loss, heat intolerance, anxiety, and tremor... read more (Graves disease) and hypothyroidism (Hashimoto thyroiditis Hashimoto Thyroiditis Hashimoto thyroiditis is chronic autoimmune inflammation of the thyroid with lymphocytic infiltration. Findings include painless thyroid enlargement and symptoms of hypothyroidism. Diagnosis... read more ).

Occasionally, vitiligo occurs after a direct physical injury to the skin (eg, as a response to sunburn). Vitiligo can also result from exposure to vitiligo-inducing phenols (1 General reference Vitiligo is a loss of skin melanocytes that causes areas of skin depigmentation of varying sizes. Cause is unknown, but genetic and autoimmune factors are likely. Diagnosis is usually clear... read more ). Patients may associate the onset of vitiligo with emotional stress.

Immunotherapy Immune Therapy Systemic cancer therapy includes chemotherapy (ie, conventional or cytotoxic chemotherapy), hormone therapy, targeted therapy, and immune therapy (see also Overview of Cancer Therapy). The number... read more for melanoma (eg, BRAF inhibitors, programmed death receptor 1 [PD-1] inhibitors) can trigger vitiligo as an immunologic side effect.

Vitiligo is characterized by hypopigmented or depigmented areas, usually sharply demarcated and often symmetric. Depigmentation may be localized, involving 1 or 2 spots or entire body segments (segmental vitiligo); rarely, it may be generalized, involving most of the skin surface (universal vitiligo). However, vitiligo most commonly involves the face (especially around the orifices), digits, dorsal hands, flexor wrists, elbows, knees, shins, dorsal ankles, armpits, inguinal area, anogenital area, umbilicus, and nipples. Cosmetic disfigurement can be especially severe and emotionally devastating in dark-skinned patients. Hair in vitiliginous areas is usually white.

Depigmented skin is typically obvious on examination, especially in darker-skinned people. Subtle hypopigmented or depigmented lesions are accentuated under a Wood light Wood light Diagnostic tests are indicated when the cause of a skin lesion or disease is not obvious from history and physical examination alone. These include Patch testing Biopsy Scrapings Examination... read more (365 nm), which shows the chalk-white appearance of depigmented skin.

Differential diagnosis includes postinflammatory hypopigmentation, piebaldism (a rare autosomal dominant disorder in which depigmented patches surrounded by hyperpigmented areas occur most often on the forehead, neck, anterior trunk, and mid-extremities), morphea (localized scleroderma, in which skin is usually sclerotic), leprosy Leprosy Leprosy is a chronic infection usually caused by the acid-fast bacilli Mycobacterium leprae or the closely related organism M. lepromatosis. These organisms have a unique tropism... read more (in which lesions are usually hypoesthetic), lichen sclerosus Lichen Sclerosus Lichen sclerosus is an inflammatory dermatosis of unknown cause, possibly autoimmune, that usually affects the anogenital area. The earliest signs are skin fragility, bruising, and sometimes... read more , pityriasis alba, chemical leukoderma, and leukoderma due to melanoma.

Although there are no evidence-based guidelines, it is reasonable for physicians to test for complete blood count, fasting blood glucose, thyroid function, and antithyroid peroxidase antibodies (frequently present in Hashimoto thyroiditis) as clinically indicated by review of systems.

Vitiligo can be challenging to manage; initial repigmentation and maintenance of pigment can be unpredictable. Physicians must be aware of individual and ethnic sensibilities regarding uniform skin coloring; the disease can be psychologically devastating. All depigmented areas are prone to severe sunburn Sunburn Sunburn is characterized by erythema and sometimes pain and blisters caused by overexposure to solar ultraviolet radiation. Treatment is similar to that for thermal burns, including cool compresses... read more and must be protected with clothing or sunscreen Prevention The skin may respond to sunlight with chronic (eg, dermatoheliosis [photoaging], actinic keratosis) or acute (eg, photosensitivity, sunburn) changes. The sun emits a wide range of electromagnetic... read more .

Small, scattered lesions may be camouflaged with makeup. With more extensive involvement, treatment is usually aimed at repigmentation. However, little is known about comparative efficacies of such treatments. Traditional first-line therapy is potent topical corticosteroids, which may also cause hypopigmentation or atrophy in normal surrounding skin as an adverse effect of chronic corticosteroid use. Calcineurin inhibitors (tacrolimus and pimecrolimus) may be particularly useful alternatives for treating areas of the skin (such as the face and groin), where adverse effects of topical corticosteroid therapy most commonly occur. Calcipotriene blended with betamethasone dipropionate may also be helpful and more successful than monotherapy with either drug.

Oral and topical PUVA are often successful, but over a hundred treatment sessions may be necessary, which can increase risk of skin cancer Overview of Skin Cancer Skin cancer is the most common type of cancer and commonly develops in sun-exposed areas of skin. The incidence is highest among outdoor workers, sportsmen, and sunbathers and is inversely related... read more . Narrowband UVB is as effective as topical PUVA and has few adverse effects, making narrowband UVB preferable to PUVA. Narrowband UVB is often the preferred initial treatment for widespread vitiligo. Excimer laser (308 nm) may be useful, particularly for localized disease that does not respond to initial topical therapy.

Surgery is reasonable only for patients with stable, limited disease when medical therapy has failed. Therapies include autologous micrografting (1 Treatment references Vitiligo is a loss of skin melanocytes that causes areas of skin depigmentation of varying sizes. Cause is unknown, but genetic and autoimmune factors are likely. Diagnosis is usually clear... read more ), suction blister grafting, and tattooing; tattooing is especially useful for difficult-to-repigment areas such as the nipples, lips, and fingertips.

Depigmentation of unaffected skin to achieve homogeneous skin tone is possible with 20% monobenzyl ether of hydroquinone applied twice a day. This treatment is indicated only when most of the skin is involved and the patient is prepared for permanent pigment loss and the subsequent increased risks of photo-induced skin damage (eg, skin cancers Overview of Skin Cancer Skin cancer is the most common type of cancer and commonly develops in sun-exposed areas of skin. The incidence is highest among outdoor workers, sportsmen, and sunbathers and is inversely related... read more , photoaging Photoaging Chronic affects of sunlight include photoaging, actinic keratoses, and skin cancer. (See also Overview of Effects of Sunlight.) Chronic exposure to sunlight ages the skin (photoaging, dermatoheliosis... read more ). This treatment can be extremely irritating, so a smaller test area should be treated before widespread use. Treatment for ≥ 1 year may be required.

The topical Janus kinase (JAK) inhibitor ruxolitinib (JAK 1 and 2) is available for the treatment of vitiligo (2 Treatment references Vitiligo is a loss of skin melanocytes that causes areas of skin depigmentation of varying sizes. Cause is unknown, but genetic and autoimmune factors are likely. Diagnosis is usually clear... read more ). Another JAK inhibitor, tofacitinib (JAK 1 and 3) is currently under investigation. However, depigmentation can recur after these agents are stopped.

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