Continuing medical educationVitiligo: A comprehensive overview: Part I. Introduction, epidemiology, quality of life, diagnosis, differential diagnosis, associations, histopathology, etiology, and work-up
Section snippets
Key points
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Vitiligo is a disorder of pigmentation manifesting as white macules and patches
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Vitiligo can occur at any age and affects both sexes equally
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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 The prevalence of vitiligo is likely less than 1%, but varies based on region 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 Vitiligo significantly impairs quality of life Women are generally more affected by the disorder than men 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 Vitiligo is classified into localized, generalized, and universal Lesions typically develop in areas of friction, reflecting koebnerization A Wood's lamp can be helpful in diagnosis; a biopsy is rarely required 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 The differential diagnosis of vitiligo is broad Occupational and iatrogenic causes of depigmentation can present like vitiligo 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 Vitiligo may be associated with other autoimmune disorders, including thyroid disease, diabetes, pernicious anemia, and psoriasis It may be associated with ophthalmologic and auditory findings 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 Histopathology can help confirm the diagnosis of vitiligo Melanocytes are absent, and there is a scant inflammatory cell infiltrate Active lesions may have a lichenoid interface dermatitis 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 The cause of vitiligo is unknown The autoimmune hypothesis is the best supported theory The neurohumoral, cytotoxic, and oxidative stress theories have moderate evidence 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.