Vitiligo Differential Diagnoses

Updated: Aug 12, 2016
  • Author: Vlada Groysman, MD; Chief Editor: Dirk M Elston, MD  more...
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DDx

Diagnostic ConsiderationsVitiligo and ocular diseaseVitiligo and autoimmune disordersVitiligo and auditory abnormalitiesVitiligo and melanoma

The uveal tract and retinal pigment epithelium contain pigment cells. Choroidal abnormalities have been reported in up to 30% of patients, and iritis has been reported in approximately 5% of patients. Exophthalmos may occur in the setting of concomitant Graves disease. Uveitis is the most significant ocular abnormality associated with vitiligo. The most severe form of uveitis is seen in the Vogt-Koyanagi-Harada syndrome. This syndrome is characterized by vitiligo, uveitis, aseptic meningitis, dysacusis, tinnitus, poliosis, and alopecia.

Alezzandrini syndrome includes facial vitiligo, poliosis, deafness, and unilateral visual changes. The affected eye has decreased visual acuity and an atrophic iris. [1]

Although the color of the irides does not change in patients with vitiligo, depigmented areas in pigment epithelium and choroid occur in up to 40% of patients.

Vitiligo is frequently associated with disorders of autoimmune origin, with thyroid abnormalities being the most common. Vitiligo usually precedes the onset of thyroid dysfunction. A study of 363 pediatric patients with both segmental and nonsegmental vitiligo was conducted at Fudan University in China. A significant incidence of thyroid dysfunction was found in patients with nonsegmental vitiligo. The authors suggested that it may be prudent to screen thyroid function and antibody levels in pediatric patients with vitiligo. [24]

Patients with autoimmune polyendocrinopathy candidiasis-ectodermal dystrophy have an increased prevalence of vitiligo. In this genetic syndrome, autoantibodies cause destruction of endocrine cells. [25]

Moreover, studies suggest that an association exists between a positive family history of vitiligo, autoimmune/endocrine diseases, leukotrichia, and an increased incidence of vitiligo in children. In addition, pediatric patients with a positive family history of vitiligo show an earlier age of disease onset. [26]

Melanin may play a significant role in the establishment and/or maintenance of the structure and function of the auditory system and may modulate the transduction of the auditory stimuli by the inner ear. [27, 28] The membranous labyrinth of the inner ear contains melanocytes, and the heaviest pigmentation is present in the scala vestibuli. Because vitiligo affects all melanocytes, auditory disturbances may result. Several studies have described familial vitiligo associated with hearing abnormalities and hypoacusis in 16% of patients younger than 40 years who have vitiligo. [28]

Vitiligolike depigmentation can occur in patients with malignant melanoma [5, 29] and is believed to result from a T-cell–mediated reaction to antigenic melanoma cells and cross-reactivity to healthy melanocytes. Most patients with melanoma or with vitiligo develop antibodies to similar antigens that are present both on melanocytes and on melanoma cells. These findings support the hypothesis that the clinical link between the 2 diseases results from immune responses to antigens shared by normal and malignant pigment cells. Studies have demonstrated that a halo nevus, hypopigmentation, or depigmentation may occur in patients with melanoma. The depigmentation or hypopigmentation spreads centrifugally from the trunk to other parts of the body. The sites of depigmentation may be remote from the original site of melanoma. Although metastasis has most likely occurred in the majority of patients, active vitiligo in these patients may signal a longer survival time than expected.

Differential Diagnoses