para>Associated with Hashimoto thyroiditis in a significant portion of children. Screening at onset and possibly annually may be beneficial.
DIAGNOSIS
HISTORY
- Inquire about recent history of sunburns, pregnancy, skin trauma, or emotional stress (1).
- Family history of premature graying, vitiligo, and autoimmune disorders
- Review of systems for related associated conditions
- Ascertain psychological impact on QOL, Dermatology Life Quality Index (DLQI).
PHYSICAL EXAM
- Full-body skin exam with Wood lamp to accentuate lesions and distinguish depigmentation from hypopigmentation (1)
- Lesions are well-demarcated, uniform white macules and patches.
- Look for evidence of repigmentation (most commonly around hair follicles).
DIFFERENTIAL DIAGNOSIS
- Infectious: tinea versicolor, leprosy, leishmaniasis, onchocerciasis, treponematoses (pinta/syphilis)
- Postinflammatory hypopigmentation: psoriasis, atopic dermatitis, pityriasis alba, systemic lupus erythematosus, scleroderma
- Inherited hypomelanoses: piebaldism, tuberous sclerosis, Waardenburg, hypomelanosis of Ito, Vogt-Koyanagi-Harada
- Malformations: nevus anemicus, nevus depigmentosus
- Paraneoplastic: mycosis fungoides, melanoma-associated leukoderma
- Occupational and chemical induced
- Occupational: phenolic/catechol derivatives and arsenic-containing compounds
- Chemical: numerous, including cosmetics, cleansers, insecticides, and even medications (imatinib, potent topical corticosteroids [TCS]) (1)
- Melasma: Normal skin may be confused as vitiligo in the setting of surrounding hyperpigmentation.
- Halo nevi
- Lichen sclerosus et atrophicus
- Idiopathic guttate hypomelanosis
- Progressive-acquired macular hypomelanosis
DIAGNOSTIC TESTS & INTERPRETATION
Initial Tests (lab, imaging)
- TSH, CBC, ANA (1)
- Consider antithyroid peroxidase, antithyroglobulin antibodies, hemoglobin, vitamin B12 levels if family/patient history of autoimmune disease (1).
Follow-Up Tests & Special Considerations
- Monitor for disease progression/flares.
- Monitor for symptoms of related conditions.
Diagnostic Procedures/Other
- Skin biopsy is rarely needed. Highest yield is with comparison of lesional/perilesional biopsies.
- Consider ophthalmologic and audiologic evaluation.
Test Interpretation
Few or no epidermal melanocytes. At margins, melanocytes may be larger, vacuolated, and dendritic. Early lesions show inflammation and later, degeneration, including of adnexa and nerves.
TREATMENT
The variant of vitiligo may affect response.
- If untreated, progression is the natural course for those with mucosal involvement, family history, koebnerization, and nonsegmental variants.
- Lesions that respond best are on the face, of recent onset, in darker skin type, and in younger patients.
GENERAL MEASURES
- Sunscreen to decrease sunburn and prevent accentuation of uninvolved skin
- Corrective camouflage as coverup (Cover FX, Dermablend)
MEDICATION
- Individualize therapy depending on age, extent, distribution, and rate of progression.
- Many therapies are considered "off-label " and not FDA-approved for vitiligo, although they are often considered first-line therapy.
- Corticosteroids: Mid-potency topical corticosteroids (mometasone furoate, fluticasone propionate) applied daily as monotherapy are considered first-line treatments. Do not use on face/axilla/groin; do not occlude except under close monitoring. Pediatric: as above, for children >12 years of age. Consider decreased potency. Local side effects including atrophy, telangiectasia, hypertrichosis, acneiform eruptions, and striae limit treatment; regular steroid holidays are recommended (2). The combination of light therapy and TCS is the most effective treatment overall (3). Most efficacious on sun-exposed areas: face/neck, dark-skinned patients, newer lesion (3). Addition of tretinoin 0.025 " 0.05% BID is effective and can decrease potential skin atrophy (4). Systemic corticosteroids can be helpful, but dosage and safety parameters have not been fully evaluated (2,5)[A].
- Topical calcineurin inhibitors: slightly inferior to TCS as monotherapy but better side effect profile (3,6,7)[B]; can be used as adjunctive to light therapy; carries a controversial black box warning for a theoretical risk of lymphoma or skin cancer. Extensive safety profiling has not revealed any evidence for this in children or adults using topical calcineurin inhibitors. Local reactions include burning sensation, pruritus, erythema, and rare transient hyperpigmentation (3).
- Tacrolimus 0.03% or 0.1% ointment BID (2). Pediatric: 0.03% ointment BID, for children >2 years of age (7)
- Pimecrolimus 1% cream BID (2). Pediatric: as adults, for children >2 years of age (7)
- Topical vitamin D3 analogs: less effective than TCS alone, but in combination with TCS or phototherapy can shorten time until, and improve stability of, repigmentation (5,7)[B].
- Calcipotriene ointment 1 to 2 times per day. Pediatric: not defined.
- Available as a combination formulation, betamethasone dipropionate 0.064%/calcipotriene 0.005% ointment daily, max dose of 100 g/week for 4 weeks, not for >30% BSA, and not for face/axilla/groin. Pediatric: not defined.
- Oral vitamin D3: reported to induce repigmentation. Oral vitamin D3 35,000 U once daily plus low-calcium diet for 6 months. Pediatric: not defined.
- Phototherapy: Narrow band UVB (NBUVB) is superior to UVA and indicated for lesions involving more than 15 " 20% BSA (5,7,8)[A]. Psoralen and khellin enhance the effect of light. Psoralen plus UVA (PUVA) may increase the incidence of skin cancers. Khellin may have reduced cross-linking of DNA and may be less carcinogenic; however, it is associated with increased liver toxicity (8). L-phenylalanine can be used topically and orally as a photosensitizer for natural or artificial light. Pediatric: Oral PUVA is contraindicated.
- Laser therapy: Excimer laser (308 nm) is superior to other light therapy. Helium " neon laser works for segmental vitiligo (7)[A].
- Antioxidants: may have protective role in preventing melanocyte degradation from reactive oxygen species. Options include vitamin C, vitamin E, Vitix, Polypodium leucotomos extracts, and Ginkgo biloba (6)[B].
- Surgical therapy: See later discussion.
- New concepts: Tumor necrosis factor-α inhibitors, cyclosporine, cyclophosphamide, azathioprine, minocycline, and immunosuppressants are currently being evaluated (8).
First Line
- Recommended: avoidance of triggering factors plus TCS alone or in combination with NBUVB
- Alternatively
- Topical calcineurin inhibitors (preferred for face, neck, axilla, and groin)
- NBUVB
- PUVA in adults
- Camouflage and psychotherapy should be offered to all patients at any stage (8).
Second Line
- Recommended: photochemotherapy with psoralens or vitamin D analogues
- Alternatively
- Topical vitamin D analogues
- Targeted phototherapy
- 308-nm laser in combination with topical steroids, topical calcineurin inhibitors, or vitamin D analogues
- Oral corticosteroids (pulse therapy)
- Surgical treatments indicated for stable 2- to 3 cm-lesions, refractory to other treatments
- Mini-punch graft (pretreat with cryotherapy/dermabrasion or posttreat with phototherapy) (9)[B]
- Suction blister graft (9)[B]
- Autologous melanocyte suspension transplant (6)[B]
ISSUES FOR REFERRAL
- Dermatologist: for facial/widespread vitiligo or when advanced therapy is necessary
- Ophthalmologist: for ocular symptoms or monitoring of TCS near eyes
- Endocrinologist: evaluation/management of associated conditions
- Psychologist: for severe distress
- Medical geneticist for associated conditions
ADDITIONAL THERAPIES
- Depigmentation therapy with monobenzone, hydroquinone, or Q-switched ruby laser: for extensive vitiligo recalcitrant to therapy (9)
- Pseudocatalase with addition of NBUVB (2)[B]
- Prostaglandin E for short-duration disease and localization to face and scalp (2)[B]
- Cosmetic tattooing for localized stable vitiligo
SURGERY/OTHER PROCEDURES
- Goal is to transport melanocytes from other areas of the skin. Methods include punch, blister, or split-thickness skin grafting, or transplantation of autologous melanocytes.
- Dermabrasion and curettage alone or in combination with 5-fluorouracil may induce follicular melanocyte reservoirs (7)[A].
- Patients who koebnerize or form keloids may be worse, and permanent scarring is a risk for all patients.
COMPLEMENTARY & ALTERNATIVE MEDICINE
- Ginkgo biloba 60 mg PO daily may significantly improve extension and spreading of lesions (8)[B].
- Polypodium leucotomos may help with repigmentation with NBUVB and aid in reducing phototoxic reactions (7,8)[B].
ONGOING CARE
FOLLOW-UP RECOMMENDATIONS
- Monitor for symptoms of related conditions.
- With topical steroids, follow at regular intervals to avoid steroid atrophy, telangiectasia, and striae distensae.
DIET
No restrictions
PATIENT EDUCATION
- Discussion of disease course, progression, and cosmesis
- Education regarding trauma/friction and Koebner phenomenon
PROGNOSIS
- Vitiligo may remain stable or slowly or rapidly progress.
- Spontaneous repigmentation is uncommon.
- Generalized vitiligo is often progressive, with flares. Focal vitiligo often has rapid onset, then stabilizes.
COMPLICATIONS
- Adverse effects of each treatment modality
- Psychiatric morbidity: depression, adjustment disorder, low self-esteem, sexual dysfunction, and embarrassment in relationships (1)
- Different cultures may have different perceptions/social stigmas about vitiligo. Some believe it to be contagious or related to infection. Women with vitiligo may have difficulty finding a marriage partner and have low self-esteem.
REFERENCES
11 Alikhan A, Felsten LM, Daly M, et al. Vitiligo: a comprehensive overview Part I. Introduction, epidemiology, quality of life, diagnosis, differential diagnosis, associations, histopathology, etiology, and work-up. J Am Acad Dermatol. 2011;65(3):473 " 491.22 Colucci R, Lotti T, Moretti S. Vitiligo: an update on current pharmacotherapy and future directions. Expert Opin Pharmacother. 2012;13(13):1885 " 1899.33 Felsten LM, Alikhan A, Petronic-Rosic V. Vitiligo: a comprehensive overview Part II: treatment options and approach to treatment. J Am Acad Dermatol. 2011;65(3):493 " 514.44 Kwon HB, Choi Y, Kim HJ, et al. The therapeutic effects of a topical tretinoin and corticosteroid combination for vitiligo: a placebo-controlled, paired-comparison, left-right study. J Drugs Dermatol. 2013;12(4):e63 " e67.55 Bacigalupi RM, Postolova A, Davis RS. Evidence-based, non-surgical treatments for vitiligo: a review. Am J Clin Dermatol. 2012;13(4):217 " 237.66 Lepe V, Moncada B, Castanedo-Cazares JP, et al. A double-blind randomized trial of 0.1% tacrolimus vs 0.05% clobetasol for the treatment of childhood vitiligo. Arch Dermatol. 2003;139(5):581 " 585.77 Whitton ME, Pinart M, Batchelor J, et al. Interventions for vitiligo. Cochrane Database Syst Rev. 2015;(2):CD003263.88 Taieb A, Alomar A, B ¶hm M, et al. Guidelines for the management of vitiligo: the European Dermatology Forum consensus. Br J Dermatol. 2013;168(1):5 " 19.99 Patel NS, Paghdal KV, Cohen GF. Advanced treatment modalities for vitiligo. Dermatol Surg. 2012;38(3):381 " 391.
CODES
ICD10
L80 Vitiligo
ICD9
709.01 Vitiligo
SNOMED
- 56727007 Vitiligo (disorder)
- 403267005 Localized vitiligo
- 403269008 Generalized vitiligo (disorder)
- 403268000 Segmental vitiligo
- 238713002 Occupational vitiligo (disorder)
- 403270009 Trichrome vitiligo (disorder)
CLINICAL PEARLS
- Vitiligo can be a psychologically devastating skin disease.
- Screen for associated diseases, particularly if onset occurs later in life.
- Treatment should be individualized based on BSA, skin type, and patient goals.
- Dermatology consultation when extensive disease, facial involvement, and when advanced treatments are considered.