Sections

Vitiligo

Presentation

History

Vitiligo presents as asymptomatic, milky white colored lesions. The lesions may be localized in focal or segmental vitiligo and generalized in non-segmental vitiligo. Universal vitiligo shows generalized depigmentation. Hairs in the affected region may be white, and this is known as leukotrichia. Patients with generalized vitiligo may present with sunburn due to inadvertent sun exposure or actinic keratosis in long-standing vitiligo. Lesions may appear at the site of trauma (koebnerization).

Physical Examination

Vitiligo is almost always diagnosed clinically upon physical examination. Vitiligo manifests as acquired depigmented macules or patches surrounded by normal skin. The macules are chalk or milk-white in color and are well demarcated. Lesions can be round, oval, or linear in shape. The borders may be convex.^[1]Lesions enlarge centrifugally over time at an unpredictable rate. Lesions range from millimeters to centimeters in size. A Wood lamp may be necessary to see lesions on patients with lighter skin. Dermoscopy may also help in the diagnosis of vitiligo. It shows diffuse white glow in established lesion; reverse pigmentary pattern in evolving vitiligo; micro-koebnerization, comet-tail like appearance in unstable vitiligo and reticular pigment network and peri-follicular pigment in stable and repigmenting vitiligo.^[18]

The most common sites of vitiligo involvement are the face, neck, forearms, feet, dorsal hand, fingers, and scalp. When found on the face, lesions may favor a periocular or perioral distribution. In the setting of widespread or generalized vitiligo, lesions may also occur around the genital region, areola, and nipple. Additionally, lesions may occur in regions frequently subjected to trauma, such as bony prominences, elbows, and knees. Koebner phenomenon is defined as the development of vitiligo in sites of trauma, such as a cut, burn, or abrasion. Koebnerization may occur in as many as 20-60% of vitiligo patients.^[19]

Body hair in vitiliginous macules may be depigmented. This is known as leukotrichia, and it may indicate a poor prognosis with regard to repigmentation therapy.^[20]Spontaneous repigmentation of depigmented hair is unlikely to occur.

Clinical Variants

Trichrome vitiligo is a clinical variant characterized by an intermediate zone of hypopigmentation located between the depigmented center and the peripheral unaffected skin. The natural evolution of the hypopigmented areas is progression to full depigmentation. This results in 3 shades of color in the same patient, as in the image below. The presentation and shades of trichrome vitiligo vary depending on the natural skin color of the patient.

Trichrome vitiligo.

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Marginal inflammatory vitiligo is a very rare variant in which a red, raised border is present at onset or may appear several months or years after initial onset. Mild pruritus may be present. See image below.

Marginal inflammatory vitiligo.

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Quadrichrome vitiligo is another variant of vitiligo, which reflects the presence of a fourth color (dark brown) at sites of perifollicular repigmentation.

Blue vitiligo is a variant of vitiligo in which a bluish tinge is seen in the depigmented macule. This bluish hue is due to the presence of dermal melanophages. ^[21]

Clinical Classifications of Vitiligo

Vitiligo may be divided into 2 groups: segmental and nonsegmental. It is important to note that other classification systems exist that choose to break down types of vitiligo based on having a localized or generalized distribution, with localized implying the lesion is restricted to a specific area and generalized implying more than one area is involved. However, the distinction between segmental and nonsegmental may be the most useful to the clinician, as it has an impact on progression, prognosis, and treatment.

Segmental vitiligo

This type manifests as 1 or more macules that may follow the lines of Blaschko. It is unilateral and does not cross the midline. Segmental vitiligo usually has an early onset and rapidly spreads in the affected area. The course of segmental vitiligo can arrest, and depigmented patches can persist unchanged for the life of the patient. This type of vitiligo is not associated with thyroid or other autoimmune disorders. See the image below.

Segmental vitiligo.

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Nonsegmental vitiligo

Nonsegmental vitiligo has served as an umbrella term to include all types of vitiligo that cannot be classified as segmental vitiligo.^[22]Of note, nonsegmental vitiligo is more strongly linked than segmental vitiligo to markers of autoimmunity or inflammation such as halo nevi and thyroid antibodies.^[23]

Examples of nonsegmental vitiligo include the following (see the image below):

Focal vitiligo: This is characterized by one or more macules in a limited area that do not follow a segmental distribution.
Generalized vitiligo: This follows a nonsegmental distribution and is more widespread than localized or focal vitiligo.

Subtypes of generalized vitiligo include the following:

Acrofacial vitiligo: Depigmentation occurs on the distal fingers and periorificial areas.
Vulgaris vitiligo: This is characterized by scattered patches that are widely distributed.
Universal vitiligo: Complete or nearly complete depigmentation of the body occurs.
Mucosal vitiligo

Nonsegmental vitiligo.

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Complications

The visual nature of the disease results in severe psychosocial and psychological consequences, especially in individuals with darker skin. It can result in social stigma and depression.

Inadvertent sun-exposure results in acute sunburn, and chronic sun damage can result in non-melanoma skin cancers

Differential Diagnoses

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Media Gallery

Trichrome vitiligo.
Marginal inflammatory vitiligo.
Segmental vitiligo.
Nonsegmental vitiligo.

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Author

Shekhar Neema, MD Associate Professor, Department of Dermatology, Armed Forces Medical College, India

Shekhar Neema, MD is a member of the following medical societies: Dermatopathology Society of India, Indian Association for the Study of Sexually Transmitted Diseases and AIDS, Indian Association of Dermatologists, Venereologists and Leprologists, International Society of Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Former Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Military Dermatologists, Phi Beta Kappa, Texas Dermatological Society

Disclosure: Nothing to disclose.

Edward F Chan, MD Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine

Edward F Chan, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Mark G Lebwohl, MD Chairman, Department of Dermatology, Mount Sinai School of Medicine

Mark G Lebwohl, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Received none from Amgen for consultant & investigator; Received none from Novartis for consultant & investigator; Received none from Pfizer for consultant & investigator; Received none from Celgene Corporation for consultant & investigator; Received none from Clinuvel for consultant & investigator; Received none from Eli Lilly & Co. for consultant & investigator; Received none from Janssen Ortho Biotech for consultant & investigator; Received none from LEO Pharmaceuticals for consultant & inves.

Barbara B Wilson, MD Edward P Cawley Associate Professor, Department of Dermatology, University of Virginia School of Medicine

Barbara B Wilson, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Medical Society of Virginia, Sigma Xi, The Scientific Research Honor Society

Disclosure: Nothing to disclose.

Vlada Groysman, MD Medical Director, Cahaba Dermatology and Skin Health Center; Clinical Assistant Professor of Dermatology, University of Alabama at Birmingham School of Medicine

Vlada Groysman, MD is a member of the following medical societies: American Academy of Dermatology, Women's Dermatologic Society, Medical Dermatology Society

Disclosure: Nothing to disclose.

Naveed Sami, MD, FAAD Assistant Professor, Department of Dermatology, University of Alabama School of Medicine

Disclosure: Nothing to disclose.

Krista Roncone University of Virginia School of Medicine

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author, Seung-Kyung Hann, MD, to the development and writing of this article.