History
The most common form of vitiligo is an amelanotic macule or patch surrounded by healthy skin. The macules are chalk or milk-white in color, and lesions are well demarcated.
The lesions are not readily apparent in lightly pigmented individuals; however, they are easily distinguishable with a Wood lamp examination.
Physical
Vitiligo manifests as acquired white or hypopigmented macules or patches. The lesions are usually well demarcated, and they are round, oval, or linear in shape. The borders may be convex.[6] Lesions enlarge centrifugally over time at an unpredictable rate. Lesions range from millimeters to centimeters in size. Initial lesions occur most frequently on the hands, forearms, feet, and face, favoring a perioral and periocular distribution.
Vitiligo lesions may be localized or generalized, with the latter being more common than the former. Localized vitiligo is restricted to one general area with a segmental or quasidermatomal distribution. Generalized vitiligo implies more than one general area of involvement. In this situation, the macules are usually found on both sides of the trunk, either symmetrically or asymmetrically arrayed.
The most common sites of vitiligo involvement are the face, neck, and scalp. Many of the most common sites of occurrence are areas subjected to repeated trauma, including the following:
- Bony prominences
- Extensor forearm
- Ventral wrists
- Dorsal hands
- Digital phalanges
Involvement of the mucous membranes is frequently observed in the setting of generalized vitiligo. Vitiligo often occurs around body orifices such as the lips, genitals, gingiva, areolas, and nipples.
Body hair (leukotrichia) in vitiliginous macules may be depigmented. Vitiligo of the scalp usually appears as a localized patch of white or gray hair, but total depigmentation of all scalp hair may occur. Scalp involvement is the most frequent, followed by involvement of the eyebrows, pubic hair, and axillary hair, respectively. Leukotrichia may indicate a poor prognosis in regard to repigmentation. Spontaneous repigmentation of depigmented hair in vitiligo does not occur.
Clinical Variants
Trichrome vitiligo has an intermediate zone of hypochromia located between the achromic 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—brown, tan, and white—in the same patient, as in the image below.
Trichrome vitiligo. Marginal inflammatory vitiligo results in a red, raised border, which is present from the onset of vitiligo (in rare cases) or which may appear several months or years after the initial onset. A mild pruritus may be present, as in the image below.
Marginal inflammatory vitiligo. Quadrichrome vitiligo is another variant of vitiligo, which reflects the presence of a fourth color (ie, dark brown) at sites of perifollicular repigmentation. A case of pentachrome vitiligo with 5 shades of color has also been described.[8]
Blue vitiligo results in blue coloration of vitiligo macules. This type has been observed in a patient with postinflammatory hyperpigmentation who then developed vitiligo.
Koebner phenomenon is defined as the development of vitiligo in sites of specific trauma, such as a cut, burn, or abrasion. Minimum injury is required for Koebner phenomenon to occur.
Clinical Classifications of Vitiligo
The classification system is important because of the special significance assigned by some authorities to each type of vitiligo. The most widely used classification of vitiligo is localized, generalized, and universal types and is based on the distribution, as follows:
Localized vitiligo
- Focal: This type is characterized by one or more macules in one area, most commonly in the distribution of the trigeminal nerve.
- Segmental: This type manifests as one or more macules in a dermatomal or quasidermatomal pattern. It occurs most commonly in children. More than half the patients with segmental vitiligo have patches of white hair or poliosis. This type of vitiligo is not associated with thyroid or other autoimmune disorders.
- Mucosal: Mucous membranes alone are affected.
Generalized vitiligo
- Acrofacial: Depigmentation occurs on the distal fingers and periorificial areas.
- Vulgaris: This is characterized by scattered patches that are widely distributed.
- Mixed: Acrofacial and vulgaris vitiligo occur in combination, or segmental and acrofacial vitiligo and/or vulgaris involvement are noted in combination.
Universal vitiligo
This is complete or nearly complete depigmentation. It is often associated with multiple endocrinopathy syndrome.
Classification of Vitiligo by Progression, Prognosis, and Treatment
When progression, prognosis, and treatment are considered, vitiligo can be classified into 2 major clinical types: segmental and nonsegmental, as demonstrated in the images below.
Segmental
This usually has an onset early in life 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.
Nonsegmental
This type includes all types of vitiligo, except segmental vitiligo.[13] See the images below.
Segmental vitiligo. 
Nonsegmental vitiligo. A single-center study of 213 patients aged 17 years or younger with segmental or nonsegmental vitiligo found that nonsegmental vitiligo was more strongly linked than segmental vitiligo to markers of autoimmunity or inflammation such as halo naevi and thyroid antibodies; patients with nonsegmental vitiligo were also more likely to have a family history of vitiligo or autoimmunity.[14]
Causes
Theories regarding destruction of melanocytes include autoimmune mechanisms, cytotoxic mechanisms, intrinsic melanocyte defects, oxidant-antioxidant mechanisms, and neural mechanisms.
- Autoimmune and cytotoxic hypotheses: Aberration of immune surveillance results in melanocyte dysfunction or destruction.
- Neural hypothesis: A neurochemical mediator destroys melanocytes or inhibits melanin production.
- Oxidant-antioxidant mechanisms: An intermediate or metabolic product of melanin synthesis causes melanocyte destruction.
- Intrinsic defect of melanocytes: Melanocytes have an inherent abnormality that impedes their growth and differentiation in conditions that support normal melanocytes.
Because none of these theories alone is entirely satisfactory, some have suggested a composite hypothesis.
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