Continuing medical education
Vitiligo: A comprehensive overview: Part I. Introduction, epidemiology, quality of life, diagnosis, differential diagnosis, associations, histopathology, etiology, and work-up

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Vitiligo is an acquired pigmentary disorder of unknown etiology that is clinically characterized by the development of white macules related to the selective loss of melanocytes. The prevalence of the disease is around 1% in the United States and in Europe, but ranges from less than 0.1% to greater than 8% worldwide. A recorded predominance of women may reflect their greater willingness to express concern about cosmetically relevant issues. Half of all patients develop the disease before 20 years of age. Onset at an advanced age occurs but is unusual, and should raise concerns about associated diseases, such as thyroid dysfunction, rheumatoid arthritis, diabetes mellitus, and alopecia areata. Generalized vitiligo is the most common clinical presentation and often involves the face and acral regions. The course of the disease is unpredictable and the response to treatment varies. Depigmentation may be the source of severe psychological distress, diminished quality of life, and increased risk of psychiatric morbidity. Part I of this two-part series describes the clinical presentation, histopathologic findings, and various hypotheses for the pathogenesis of vitiligo based on past and current research.

Section snippets

Key points

  • Vitiligo is a disorder of pigmentation manifesting as white macules and patches

  • Vitiligo can occur at any age and affects both sexes equally

  • Vitiligo is typically asymptomatic

Vitiligo is an acquired disorder of the skin and mucous membranes that is characterized by well circumscribed, depigmented macules and patches and that occurs secondary to selective destruction of melanocytes.1, 2 It may appear at any age; cases have been reported as early as 6 weeks after birth.1, 3, 4 Approximately 0.5% to

Epidemiology

Key points

  1. The prevalence of vitiligo is likely less than 1%, but varies based on region

  2. Females usually acquire the disease earlier than males

The published prevalence of vitiligo is 0.5% to 1%.7 Large studies in China, India, and Denmark have found the prevalence to be 0.093%, 0.005%, and 0.38%, respectively.11, 12, 13 Gujarat, India is considered to have the highest prevalence in the world, at about 8.8%.14 Men and women are equally affected,13, 15 but women are more likely to seek treatment.16, 17

The

Quality of life

Key points

  1. Vitiligo significantly impairs quality of life

  2. Women are generally more affected by the disorder than men

  3. It is important to assess a patient’s quality of life during encounters

Vitiligo is a psychologically devastating disorder. The fact that it typically occurs in exposed areas (the face and hands) has a major impact on self-esteem and perception of self. In many societies, vitiligo is poorly understood and is believed to be a sign of leprosy or sexually transmitted infection. In these

Diagnosis

Key points

  1. Vitiligo is classified into localized, generalized, and universal

  2. Lesions typically develop in areas of friction, reflecting koebnerization

  3. A Wood's lamp can be helpful in diagnosis; a biopsy is rarely required

  4. Rare types of vitiligo include ponctué and quadrichrome

Classically, discrete, uniformly white macules or patches with convex borders are surrounded by normal skin (Fig 4).46 Though typically asymptomatic, itch has been reported.47, 48 Vitiligo frequently occurs at sites that are normally

Differential diagnosis

Key points

  1. The differential diagnosis of vitiligo is broad

  2. Occupational and iatrogenic causes of depigmentation can present like vitiligo

  3. Common disorders with similar presentation include nevus depigmentosus, idiopathic guttate hypomelanosis, and tinea versicolor

The differential diagnosis of vitiligo is broad (Table II); however, good history taking, a thorough physical examination, and the judicious use of histopathology generally yields a straightforward diagnosis (Fig 8).

Chemical leukoderma and

Associations and syndromes

Key points

  1. Vitiligo may be associated with other autoimmune disorders, including thyroid disease, diabetes, pernicious anemia, and psoriasis

  2. It may be associated with ophthalmologic and auditory findings

  3. It can be a part of several syndromes, including autoimmune polyendocrinopathy-candidiasis-ectodermal dysplasia and Schmidt syndrome

Vitiligo may be associated with many primarily AI disorders (Table III). The link with AI thyroid disorders (hypothyroidism and hyperthyroidism) is the most well established.

Histopathology

Key points

  1. Histopathology can help confirm the diagnosis of vitiligo

  2. Melanocytes are absent, and there is a scant inflammatory cell infiltrate

  3. Active lesions may have a lichenoid interface dermatitis

  4. Immunohistochemical staining verifies the complete absence of melanocytes in skin that may still have melanin granules within keratinocytes

Histopathologic evaluation may help differentiate vitiligo from other disorders in ambiguous cases.110 Vitiligo lesions typically appear unremarkable with only scant cellular

Etiology

Key points

  1. The cause of vitiligo is unknown

  2. The autoimmune hypothesis is the best supported theory

  3. The neurohumoral, cytotoxic, and oxidative stress theories have moderate evidence

  4. New theories focus on melanocytorrhagy and decreased melanocyte survival

It remains unclear what causes damage to melanocytes and their subsequent disappearance in affected skin. There are several pathophysiologic theories; the most prominent are autoimmune, neurohumoral, and autocytotoxic. None are mutually exclusive, and it is

Summary of etiologic theories

The cause of vitiligo still remains unknown, although it is clear that several different pathophysiologic processes may be involved. The autoimmune hypothesis is best supported because of the numerous genetic association and genetic linkage studies, in combination with humoral and cellular immune aberrancies. The neurohumoral, cytotoxic, and oxidative stress theories have moderate evidence. Newer theories, such as melanocytorrhagy and decreased melanocyte survival, are just beginning to accrue

Work-up recommendations

In a patient with new-onset depigmentation, a thorough history and physical examination will usually establish the diagnosis of vitiligo; examination with a Wood's lamp will help determine true extent of involvement regardless of skin type (Fig 8). In cases where the diagnosis is less obvious, histopathologic evaluation is typically diagnostic. Specimens should be obtained both from lesional and normal skin if possible, because comparing the two may yield a higher diagnostic accuracy. Screening

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    Drs Alikhan and Felsten contributed equally to this manuscript.

    Funding sources: None.

    Reprints not available from the authors.

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