About Vitiligo

Vitiligo
Last Updated: October 9, 2001 Rate this Article
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Synonyms and related keywords: white spot disease

AUTHOR INFORMATION Section 1 of 10
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Author: Seung-Kyung Hann, MD, Associate Professor, Department of Dermatology, Severance Hospital, Yonsei University College of Medicine, Korea

Seung-Kyung Hann, MD, is a member of the following medical societies: American Academy of Dermatology

Editor(s): Mark G Lebwohl, MD, Professor and Chair, Department of Dermatology, Mount Sinai School of Medicine; David F Butler, MD, Professor, Texas A&M University College of Medicine; Director, Division of Dermatology, Scott and White Clinic; Edward F Chan, MD, Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University; and Dirk M Elston, MD, Consulting Staff, Department of Dermatology, Geisinger Medical Center
INTRODUCTION Section 2 of 10
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Background About Vitiligo : it is a specific type of leukoderma characterized by depigmentation of the epidermis. Occasionally, the loss of melanin (ie, hypopigmentation) is partial. It is an acquired progressive disorder in which some or all of the melanocytes residing in the interfollicular epidermis and, occasionally, in the hair follicles are selectively destroyed.

Pathophysiology: Mechanisms by which the melanocytes are lost may be multiple but are not yet identified unequivocally.

Frequency:

Internationally: Vitiligo is relatively frequent, at 1-2%. In about 30% of patients, there is familial clustering of cases. Some observations seem to support autosomal dominant inheritance with variable expression and incomplete penetrance. The disease itself is not inherited, but the disposition to have vitiligo is inherited. HLA antigens do not show consistent associations, ie, increased human leukocyte antigen DR4 (HLA-DR4) in blacks, human leukocyte antigen B13 (HLA-B13) in Moroccan Jews, and human leukocyte antigen B35 (HLA-B35) in Yemenite Jews. Association with HLA-B13 was described in the presence of antithyroid antibodies.
Sex: There is no sex-related difference in prevalence.

Age: Vitiligo may appear from birth to senescence, although onset most commonly is in persons aged 10-30 years.

It rarely is seen in infancy or old age. Nearly all vitiligo is acquired relatively early in life.
Average age of onset is around 20 years. It seems unlikely that the age of onset varies between the sexes.
Heightened concern about the appearance of the skin may contribute to an early awareness of the condition among women.
CLINICAL Section 3 of 10
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History:

In early vitiligo, white color areas are not distinct and may be pruritic.
Primarily, vitiligo progresses without any symptoms.
In late vitiligo, tendency of spreading can be stopped.
Physical:

Vitiligo appears as sharply circumscribed, cosmetically disturbing, white spots that stand out particularly prominently when the skin is tanned because of the contrast.
At first, there are usually only a few, small, sharply, circumscribed foci, the borders of which often are hyperpigmented.
Lesions increase in number, can become confluent, and then take on bizarre shapes.
Vitiligo lesions may be localized or generalized, the latter being more common.

Localized vitiligo is restricted to one general area including a segmental or quasi-dermatomal distribution.

Generalized vitiligo implies more than one general area of involvement; the macules usually are found on both sides of the trunk either symmetrically or asymmetrically arrayed.
Most common sites of involvement are the face, neck, and scalp.
Many of the most common sites of occurrence are areas subjected to repeated trauma, to include the following:

Bony prominences

Extensor forearm

Ventral wrists

Dorsal hands (About Vitiligo)

Digital phalanges
Mucus membrane involvement frequently is encountered in the setting of generalized vitiligo.
Vitiligo quite often occurs around body orifices such as the lips, genitals, gingiva, areolae, and nipples.
Depigmentation of body hair in vitiliginous macules may be present.

Vitiligo of the scalp usually presents 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 eyebrow, pubic, and axillary hair, respectively.

Leukotrichia may be a marker for poor prognosis in regards to repigmentation.
Clinical classification of vitiligo: The classification system is important because of the special significance assigned by some authorities to each type of vitiligo. The widely-used classification of vitiligo into localized, generalized, and universal types is based on the distribution, which is as follows:
Localized

Focal - One or more macules in one area but not clearly in a segmental or zosteriform distribution

Segmental - One or more macules in a quasi-dermatomal pattern

Vitiligo Treatment

Mucosal - Mucus membrane alone
Generalized

Acrofacial - Distal extremities and face

Vulgaris - Scattered macules

Mixed - Acrofacial and vulgaris, or segmental and acrofacial and/or vulgaris

Universal - Complete or nearly complete depigmentation
Considering the aspects of progression, prognosis, and treatment, vitiligo also can be classified into 2 major clinical types, segmental and nonsegmental.

Segmental vitiligo usually has an onset early in life and spreads rapidly within the affected area.

The course of segmental vitiligo can arrest and depigmented patches can persist unchanged for the life of patient (see Picture 1).

The nonsegmental type includes all types of vitiligo except segmental vitiligo (see Picture 2).
Causes: Four pathogenic theories have been discussed, as follows:

Immune hypothesis: Aberration of immune surveillance results in melanocyte dysfunction or destruction.
Neural hypothesis: A neurochemical mediator destroys melanocytes or inhibits melanin production.
Self-destruction hypothesis: An intermediate or metabolic product of melanin synthesis causes melanocyte destruction.
Genetic hypothesis: 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.
DIFFERENTIALS Section 4 of 10
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Piebaldism
Tinea Versicolor
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WORKUP Section 5 of 10
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Lab Studies:

Although the diagnosis of vitiligo generally is made clinically, a biopsy occasionally may be helpful to differentiate it 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 onset of hypothyroidism, diabetes, or other autoimmune disease. If signs or symptoms occur, appropriate tests should be performed.
Thyroid-stimulating hormone (TSH) test is the most cost-effective screening test for thyroid disease.
Screening for diabetes can be accomplished with a fasting blood sugar or glycosylated hemoglobin.
Other Tests:

Diagnosis is made by inspection with a Wood lamp.
TREATMENT Section 6 of 10
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Medical Care:

No single therapy for vitiligo predictably produces good results in all patients; response to therapy is highly variable.
Treatment must be individualized, and patients should be made aware of the risks associated with therapy.
Systemic photochemotherapy induces cosmetically satisfactory repigmentation in up to 70% of early or localized cases.
PUVA treatment (8-methoxypsoralen, 5-methoxypsoralen, trimethylpsoralen plus UVA) is often the most practical choice for treatment, especially in widespread vitiligo in patients with skin types IV-VI. However, 2 or 3 treatments per week for many months are required before repigmentation from perifollicular openings merges to produce confluent repigmentation.

The best results can be obtained on the face and on the proximal parts of extremities.

Vitiligo on the back of hands and feet is very resistant to therapy.
Systemic steroids (prednisone) have been used, although their prolonged use and toxicity make them undesirable.

Steroids have been given with anecdotal success in pulse doses or low doses to minimize adverse effects.

Benefits and toxicity of this therapy must be weighed carefully.

More research is necessary to establish safety and effectiveness of this therapy for vitiligo.

A topical steroid preparation often is chosen first to treat localized vitiligo because it is easy and convenient for both doctors and patients to maintain the treatment.

Results of therapy have been reported as moderately successful, particularly in patients with localized vitiligo and/or an inflammatory component to their vitiligo, even if inflammation is subclinical.
If vitiligo is widespread and attempts at repigmentation do not produce satisfactory results, depigmentation may be attempted in selected patients.

Long-term social and emotional consequences of depigmentation must be considered.

Depigmentation should not be attempted unless the patient fully understands that the procedure generally results in permanent depigmentation.

Some authorities have recommended consultation with a mental health professional to discuss potential social consequences of dipigmentation.

A 20% cream of monobenzylether of hydroquinone is applied twice daily for 3-12 months. Burning or itching may occur. Allergic contact dermatitis may be seen.

Topical PUVA is of benefit in some patients with localized lesions. Cream and solution of 8-methoxypsoralen (0.1-0.3% concentration) are available for this treatment.

It is applied 30 minutes prior to UV-A radiation (usually 0.1-0.3 J/cm2 UV-A). It should be applied once or twice a week.

Physicians who prescribe PUVA therapy should be thoroughly familiar with the risks associated with the treatment.

Additional UV-A exposure should be avoided while skin is sensitized.

Severe burns may occur if patients receive additional UV-A exposure.

Sunscreens should be given to all patients with vitiligo to minimize risk of sunburn or repeated solar damage to depigmented skin.

Patients must understand that most sunblocks have a limited ability to screen UV-A light.

Tanning of surrounding normal skin exaggerates the appearance of vitiligo, and this is prevented by sun protection. Sunscreens with a sun protection factor (SPF) of 15 or higher are best.
Surgical Care: Patients who have small areas of vitiligo with stable activity are candidates for surgical transplants.

Punch grafts
Punch biopsy specimens from a pigmented donor site are transplanted into depigmented sites.
Repigmentation and spread of color begin about 4-6 weeks post graft.
The major problem is a residual, pebbled, pigmentary pattern.
Minigrafts
Small donor grafts are inserted into the incision of recipient sites and held in place by a pressure dressing.
The graft heals readily and begins to repigment within 4-6 weeks.

Some pebbling persists but is minimal, and the cosmetic result is excellent.
Suction blister

Epidermal grafts can be obtained by vacuum suction usually with 150 mm Hg.

The recipient site can be prepared by suction, freezing, or dermabrasion of the sites, 24 hours prior to grafting.

The depigmented blister roof is discarded, and the epidermal donor graft is placed on the vitiliginous areas.
Autologous cultures and autologous melanocytes grafts: These 2 techniques are expensive and currently impractical.
Micropigmentation

Tattooing can be used to repigment depigmented skin in dark-skinned individuals.

Color matching is difficult, and the color tends to fade. Skin can be dyed with dihydroxyacetone preparations, although the color match is often poor.

MEDICATION Section 7 of 10
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The goals of pharmacotherapy are to reduce morbidity and to prevent complications.

Drug Category: Corticosteroids -- Have anti-inflammatory properties and cause profound and varied metabolic effects. In addition, these agents modify the body's immune response to diverse stimuli. Used to stop spreading and accomplish repigmentation. Data supporting efficacy of such treatment is largely anecdotal. More study is needed to establish safety and efficacy of systemic agents.Drug Name
Triamcinolone (Aristocort) -- For inflammatory dermatosis responsive to steroids; decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing capillary permeability. Intramuscular injection may be used for widespread skin disorder or intralesional injections may be used for localized skin disorder. Corticosteroid with moderately high potency; available as both ointment (0.1%) and cream (0.5%).
Adult Dose Apply a thin film qd; more frequent applications may be required, especially in areas where preparation tends to be removed before absorption is complete
Pediatric Dose Apply as in adults
Contraindications Documented hypersensitivity; fungal, viral, and bacterial skin infections
Interactions Coadministration with barbiturates, phenytoin, and rifampin decreases effects of triamcinolone
Pregnancy C - Safety for use during pregnancy has not been established.
Precautions Do not use in decreased skin circulation; prolonged use, applications over large areas, and use of potent steroids and occlusive dressings may result in systemic absorption; systemic absorption may cause Cushing syndrome, reversible HPA axis suppression, hyperglycemia, and glycosuria
Drug Name
Hydrocortisone (Westcort) -- 0.2%. Adrenocorticosteroid derivative suitable for application to skin or external mucous membranes. Has mineralocorticoid and glucocorticoid effects resulting in anti-inflammatory activity.
Adult Dose Apply sparingly to affected areas tid
Pediatric Dose Apply as in adults
Contraindications Documented hypersensitivity; viral, fungal, and bacterial skin infections
Interactions None reported
Pregnancy C - Safety for use during pregnancy has not been established.
Precautions Prolonged use, applying over large surface areas, application of potent steroids, and occlusive dressings may increase systemic absorption of corticosteroids and may cause Cushing syndrome, reversible HPA axis suppression, hyperglycemia, and glycosuria
Drug Name
Prednisone (Deltasone) -- Immunosuppressant for treatment of autoimmune disorders; may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Stabilizes lysosomal membranes and also suppresses lymphocytes and antibody production.
Use for 8 wk with taper.
Adult Dose 20 mg PO qd for 4 wk; then 10 mg PO qd for 4 wk; taper over 2 wk, as symptoms resolve
Pediatric Dose 4-5 mg/m2/d PO; alternatively, 0.05-2 mg/kg PO divided bid/qid; taper over 2 wk, as symptoms resolve
Contraindications Documented hypersensitivity; viral infection; peptic ulcer disease; hepatic dysfunction; connective tissue infections; fungal or tubercular skin infections; GI disease
Interactions Coadministration with estrogens may decrease prednisone clearance; when used with digoxin, digitalis toxicity secondary to hypokalemia may increase; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics
Pregnancy B - Usually safe but benefits must outweigh the risks.
Precautions Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use
Drug Category: Psoralens -- Used with UV-A exposure for treatment of localized vitiligo. When used systemically, used with UV-A exposure for treatment of generalized vitiligo.Drug Name
Methoxsalen (8-MOP, Oxsoralen) -- Inhibits mitosis by binding covalently to pyrimidine bases in DNA when photoactivated by UV-A. Effective in the treatment of hyperkeratosis.
Adult Dose Oral: 0.3-0.4 mg/kg PO with food 1.5 h prior to UV-A exposure, once or twice weekly; alternatively, 0.57 mg/kg 1.5-2 h before exposure to UV light, at least 48 h apart
0.1% ointment: UV-A exposure in controlled fashion 30 min after ointment application once or twice weekly
Pediatric Dose <12 years: Not recommended
>12 years: Administer as in adults
Contraindications Documented hypersensitivity; squamous cell cancer; cataract; light sensitive diseases (eg, lupus, porphyria); ingestion of photosensitizing drugs; hepatitic disease; arsenic therapy; inability to comply with instructions or unwillingness to use protective glasses following treatment
Interactions Toxicity increases with phenothiazines, griseofulvin, nalidixic acid, tetracyclines, thiazides, and sulfanilamide
Pregnancy C - Safety for use during pregnancy has not been established.
Precautions Prolonged use with UV-A causes photoaging of skin; phototoxic reactions possible; caution with hepatic insufficiency; eye protection necessary during and after therapy; use only if response to other forms of therapy is inadequate; long-term use may increase risk of skin cancer
Drug Name
Trioxsalen (Trisoralen) -- For the treatment of hyperkeratosis. In presence of UV-A radiation, inhibits mitosis by covalently binding to pyrimidine bases in DNA.
Adult Dose 0.6-0.8 mg/kg PO with food 1.5 h prior to UV-A exposure, once or twice weekly; alternatively,
10 mg/d once, 2-4 h before controlled exposure to UV-A or sunlight; not to exceed 14 d
Pediatric Dose <12 years: Not recommended
>12 years: Administer as in adults
Contraindications Documented hypersensitivity; a history of melanoma, acute lupus erythematosus, and porphyria; inability to comply with instructions regarding UV-A exposure and eye protection
Interactions None reported
Pregnancy C - Safety for use during pregnancy has not been established.
Precautions Prolonged use with UV-A causes photoaging of skin; severe burns may occur from sunlight or UV-A exposure if dose or frequency is exceeded; caution with hepatic insufficiency
MISCELLANEOUS Section 8 of 10
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Medical/Legal Pitfalls:

Physicians who prescribe PUVA therapy should be thoroughly familiar with the risks of burns, cataract formation, and carcinogenesis associated with treatment.
PICTURES Section 9 of 10
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Caption: Picture 1. Segmental vitiligo
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Caption: Picture 2. Nonsegmental vitiligo
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BIBLIOGRAPHY Section 10 of 10
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Arndt KA, Le Boit PE, Robinson JK, et al: Vitiligo. Cutaneous Medicine and Surgery 12: 1210-1218.
Burton JL, Champion RH, Breathnach SM, Burn DA: Vitiligo. In: Textbook of Dermatology. 6th ed. Blackwell Science Inc; 1998: 1802-1805.
Hann SK, Park YK, Chun WH: Clinical features of vitiligo. Clin Dermatol 1997 Nov-Dec; 15(6): 891-7[Medline].
Moscher DB: Vitiligo. In: Fitzpatrick T, Austen K, Eisen A, et al, eds. Dermatology in General Medicine. 4th ed. McGraw-Hill Health Professions Division; 1993: 923-933.
Nordlund JJ, Ortonne JP: Vitiligo vulgaris. In: King R, Nordlund J, Boissy R, Hearing V, eds. The Pigmentary System: Physiology & Pathophysiology. Oxford University Press Inc; 1998: 513-540.

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