eMedicine Specialties > Dermatology > Diseases of Pigmentation
Vitiligo: Differential Diagnoses & Workup
Updated: Aug 8, 2008
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Differential Diagnoses
| Addison Disease | Postinflammatory depigmentation |
| Alezzandrini Syndrome | Prior treatment with corticosteroids |
| Chemical leukoderma | Scleroderma |
| Halo Nevus | Tinea Versicolor |
| Idiopathic Guttate Hypomelanosis | Treponematosis |
| Leprosy | Tuberous Sclerosis |
| Malignant Melanoma | Vogt-Koyanagi-Harada Syndrome |
| Nevus Anemicus | Waardenburg Syndrome |
| Onchocerciasis (River Blindness) | |
| Piebaldism | |
| Pityriasis Alba |
Other Problems to Be Considered
Vitiligo and ocular disease
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.6
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 and autoimmune disorders
Vitiligo is frequently associated with disorders of autoimmune origin, with thyroid abnormalities being the most common. Vitiligo usually precedes the onset of thyroid dysfunction. Patients with autoimmune polyendocrinopathy candidiasis-ectodermal dystrophy have an increased prevalence of vitiligo. In this genetic syndrome, autoantibodies cause destruction of endocrine cells.11
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.12
Vitiligo and auditory abnormalities13
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.14 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.14
Vitiligo and melanoma15,16
Vitiligolike depigmentation can occur in patients with malignant melanoma 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.
Workup
Laboratory Studies
Although the diagnosis of vitiligo generally is made on the basis of clinical findings, biopsy is occasionally helpful for differentiating vitiligo from other hypopigmentary disorders.
Vitiligo may be associated with other autoimmune diseases, especially thyroid disease and diabetes mellitus. Other associated autoimmune diseases include pernicious anemia, Addison disease, and alopecia areata. Patients should be made aware of signs and symptoms that suggest the onset of hypothyroidism, diabetes, or other autoimmune disease. If signs or symptoms occur, appropriate tests should be performed.16
- Thyrotropin testing is the most cost-effective screening test for thyroid disease. Antinuclear antibody screening is also helpful. A CBC count with indices helps rule out anemia.
- Clinicians should also consider investigating for serum antithyroglobulin and antithyroid peroxidase antibodies, particularly if thyroid involvement is suspected. Antithyroid peroxidase antibodies are regarded as a sensitive and specific marker of autoimmune thyroid antibodies. Screening for diabetes can be accomplished with fasting blood glucose or glycosylated hemoglobin testing.
Other Tests
Vitiligo is diagnosed by means of inspection with a Wood lamp.
Histologic Findings
Microscopic examination of involved skin shows a complete absence of melanocytes in association with a total loss of epidermal pigmentation. Superficial perivascular and perifollicular lymphocytic infiltrates may be observed at the margin of vitiliginous lesions, consistent with a cell-mediated process destroying melanocytes. Degenerative changes have been documented in keratinocytes and melanocytes in both the border lesions and adjacent skin. Other documented changes include increased numbers of Langerhans cells, epidermal vacuolization, and thickening of the basement membrane. Loss of pigment and melanocytes in the epidermis is highlighted by Fontana-Masson staining and immunohistochemistry testing.17,18
More on Vitiligo |
| Overview: Vitiligo |
 Differential Diagnoses & Workup: Vitiligo |
| Treatment & Medication: Vitiligo |
| Follow-up: Vitiligo |
| Multimedia: Vitiligo |
| References |
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References
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Further Reading
Keywords
vitiligo, hypopigmentation, white spot disease, acquired leukoderma, nonpigmented skin, depigmented skin, depigmentation, loss of melanin, hypomelanosis, HLA-DR4, HLA-B13, HLA-B35, leukotrichia, trichrome vitiligo, blue vitiligo
