Vitiligo – 10 Interesting Facts

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What is Vitiligo

Interesting Facts

  1. Vitiligo is a common chronic condition that causes melanocyte destruction and subsequent patchy depigmentation of the skin and hair follicles
  2. 2 main classifications of disease are vitiligo/nonsegmental vitiligo (more generalized and common form) and segmental vitiligo (less common), differing primarily by their distribution patterns and tendency to progress 1
  3. Caused by a combination of genetic and environmental factors, it is sometimes associated with family history of vitiligo and family or personal history of autoimmune disease
  4. Diagnosis is based on patient history and physical examination 2
  5. Initial treatment is based on type of vitiligo and body surface area involved; all patients should avoid triggers such as sunburn and skin trauma 3
  6. Phototherapy with narrow band UV-B light, is considered the gold standard therapy in most patients unless involved area is very limited
  7. Topical corticosteroids or calcineurin inhibitors are first line therapy when limited body surface area is involved. Calcineurin inhibitors are preferred for the face and other thin-skinned areas; these are often combined with phototherapy
  8. Surgery is reserved for patients with stable, recalcitrant lesions
  9. Depigmentation therapy is reserved for patients with vitiligo universalis and others with significant vitiligo whose condition does not respond to repigmentation efforts
  10. Cosmetic camouflage (if desired) and counseling are recommended for all patients to help cope with psychosocial burden of disease 4

Pitfalls

  • On thin-skinned areas such as the face, topical calcineurin inhibitors are preferable to potent corticosteroids, as they are less likely to cause adverse effects (eg, atrophy)

Terminology

Clinical Clarification

  • Vitiligo is a chronic skin condition caused by destruction of melanocytes, resulting in patchy depigmentation of skin and hair follicles 2
  • Presents as well-circumscribed white macules and patches; in the most common form of vitiligo, these are bilateral and relatively symmetrical in location
  • May be associated with personal or family history of autoimmune disease, most commonly autoimmune thyroid disease

Classification

  • Vitiligo/nonsegmental vitiligo (accounts for nearly 80% of all cases 5)
    • Terminology for this classification is under discussion 1
      • Older terminology was nonsegmental vitiligo, primarily to differentiate from segmental vitiligo
      • New terminology for this classification should eventually be vitiligo, according to 2012 Vitiligo Global Issues Consensus Conference 1
      • This consensus recommended using the encompassing term, vitiligo/nonsegmental vitiligo until new terminology becomes commonly used; in practice, this classification is often still referred to as nonsegmental vitiligo
    • Presentations 1
      • Generalized vitiligo (most common)
        • Depigmented macules and patches involving both sides of the body, typically in a relatively symmetrical pattern; may involve oral and genital mucous membranes
      • Acrofacial vitiligo
        • Depigmented macules and patches limited to face, hands, head, and feet
      • Mucosal vitiligo
        • Depigmented macules and patches limited to the oral or genital mucosa (more than 1 site)
      • Vitiligo universalis
        • Complete or near-complete (80%-90% of body surface 1) depigmentation of the skin
  • Segmental vitiligo (5%-10% of all cases 6)
    • Characterized by segmental and unilateral distribution of cutaneous macules or patches 1
  • Mixed vitiligo 1
    • Characterized by lesions of both segmental vitiligo and vitiligo/nonsegmental vitiligo
    • The following criteria are used to identify mixed vitiligo:
      • Absence of depigmented areas in a segmental distribution at birth and during first year of life
      • Segmental vitiligo that affects at least 20% of the theoretical distribution of a dermatomal segment or presenting a definite Blaschko line distribution 1
      • Occurrence of vitiligo/nonsegmental vitiligo at least 6 months after occurrence of segmental vitiligo
      • Response to narrow band UV-B treatment
        • Poor response of segmental vitiligo
        • Good response of vitiligo/nonsegmental vitiligo
      • Exclusion of nevus depigmentosus by Wood lamp examination
  • Unclassified vitiligo 1
    • Early disease that cannot be clearly placed into the vitiligo/nonsegmental vitiligo or segmental vitiligo groups with initial presentation
    • Mucosal vitiligo
      • Limited to either oral or genital mucosa (1 site)
    • Focal vitiligo
      • Characterized by isolated patches of depigmentation that do not fit the pattern of segmental vitiligo
      • Fails to progress to vitiligo/nonsegmental vitiligo after a period of 2 years 1
    • Rare forms
      • Vitiligo punctata: characterized by sharply demarcated, punctiform macules 1 to 1.5 mm in size occurring on any area of the body 1
      • Follicular vitiligo: primarily involves follicular reservoir, with whitening of most body hairs

Diagnosis

Clinical Presentation

  • From Rodrigues M et al: New discoveries in the pathogenesis and classification of vitiligo. J Am Acad Dermatol. 77(1):1-13, 2013, Figure 2.Lip-tip variant of acrofacial vitiligo in a young African girl.
  • From Goh B-K et al: Presentations, signs of activity, and differential diagnosis of vitiligo. Dermatol Clin. 35(2):135-44, 2017, Figure 1.Vitiligo lesion showing clinical signs of rapid progression. – Confettilike depigmentation, trichrome vitiligo, and Koebner phenomenon (linear depigmentation at site of scratching) are visible.
  • From Rodrigues M et al: New discoveries in the pathogenesis and classification of vitiligo. J Am Acad Dermatol. 77(1):1-13, 2017, Figure 4.Segmental variant of vitiligo. – Note block-shaped area of depigmentation.
  • From Rodrigues M et al: New discoveries in the pathogenesis and classification of vitiligo. J Am Acad Dermatol. 77(1):1-13, 2017, Figure 6.Trichrome pattern on the neck and shoulders.
  • From Passeron T et al: Physiopathology and genetics of vitiligo. J Autoimmunity. 25(Suppl):63-8, 2005, Figure 1.Nonsegmental vitiligo in a symmetrical distribution.
  • From Hawryluk EB et al: Pediatric melanoma, moles, and sun safety. Pediatr Clin North Am. 61(2):279-91, 2014, Figure 5.Halo nevus.
  • From Rodrigues M et al: Current and emerging treatments for vitiligo. J Am Acad Dermatol. 77(1):17-29, 2017, Figure 8.Lesion on left upper eyelid with poliosis.
  • From Nicolaidou E et al: Pigmentation disorders: hyperpigmentation and hypopigmentation. Clin Dermatol. 32(1):66-72, 2014, Figure 5.Nonsegmental vitiligo on the face.
  • From van Geel N et al: Segmental vitiligo. Dermatol Clin. 35(2):145-50, 2017, Figure 1.(A) Distribution patterns of segmental vitiligo on the face. (B) Distribution patterns of segmental vitiligo on the trunk.
  • From Taïeb A et al: Special considerations in children with vitiligo. Dermatol Clin. 35(2):229-33, 2017, Figure 1.Segmental vitiligo, note sharp midline demarcation.

History

  • Personal history of autoimmune or inflammatory disease in up to 25% of patients 7
    • Most common: autoimmune thyroid disease
    • Less common
      • Type 1 diabetes mellitus
      • Pernicious anemia
      • Rheumatoid arthritis
      • Alopecia areata
      • Atopic dermatitis
      • Addison disease
      • Psoriasis
      • Dermatitis herpetiformis
      • Celiac disease
      • Systemic lupus erythematous
      • Sjögren syndrome
      • Myasthenia gravis
      • Inflammatory bowel disease
      • Sensorineural or ocular disease
  • Family history of vitiligo or other autoimmune diseases may be present 8
  • May report occupational contact with phenols (or similar chemicals) and depigmentation starting at sites of exposure, with later progression to other body sites 2
  • Onset is insidious with appearance of asymptomatic hypopigmented or depigmented patches of skin or hair
  • Sites of involvement and speed of progression depend on specific type of vitiligo 9
    • Vitiligo/nonsegmental vitiligo 10
      • Face is most common site affected, particularly eyelids and neck; also affects limbs and trunk 9
      • Lesions may appear on areas exposed to chronic trauma or rubbing 10
      • Usually progresses slowly over time, with centrifugal expansion and/or appearance of new lesions over years
    • Segmental vitiligo
      • Face is most common site affected (often the trigeminal dermatome), followed by trunk, neck, and limbs 9
      • Lesions typically spread rapidly within a segment, then stabilize without further spread (most commonly stabilized within 1 year of onset) 1 11
      • Early development of white hair patches (poliosis) in affected skin areas is a hallmark 2 (up to 50% of patients 1)
  • Presentation in children is somewhat different 9
    • First sites of involvement are often knees, elbows, shins, arms, and hands
    • Perianal region and buttocks are common sites of onset in toddlers

Physical examination

  • Full body examination is needed to identify all affected areas; inspection under Wood lamp may be helpful
    • Vitiligo presents as white, nonscaly macules and patches with distinct borders surrounded by normal skin 10
      • Appearance of individual macules/patches
        • In segmental vitiligo, often blocklike or bandlike in shape; in vitiligo/nonsegmental vitiligo, tends to have more rounded edges
        • Depigmented areas may range from several millimeters to several centimeters in diameter 12
        • Halo nevi (circumferential area of depigmented skin surrounding pigmented nevus) are common (8-10 times more common in patients with vitiligo than in general population) 13
        • Examination with Wood lamp will reveal chalky white enhancement of depigmented skin (no enhancement of hypopigmented skin) 2
          • Fair-skinned patients may require Wood light examination to confirm depigmentation
      • Distribution of macules/patches 12
        • In vitiligo/nonsegmental vitiligo, affected areas are bilateral and relatively symmetrical
        • In segmental vitiligo, depigmented patches will be unilateral without crossing the midline; there may be a single unique patch, or several patches
          • Although often described as being in dermatomal distribution, observational studies do not corroborate a strict anatomic correlation 11
        • Commonly affected areas
          • Face, especially periorofacial areas
          • Limbs, especially dorsum of hands, flexor aspect of wrists, dorsal ankle, shins, elbows, and knees
          • Trunk, especially nipples, umbilicus, axillae, sacral area, inguinal folds, and anogenital area
          • White hair may overlie depigmented skin
          • Periungual plus lip involvement may be seen in acrofacial vitiligo
    • Clinical markers of active, progressive disease include: 2
      • Koebner phenomenon
        • Development of new depigmentation at sites of previously uninvolved, recently traumatized skin 6
      • Trichrome lesions 2
        • Characterized by development of a ring of hypopigmented skin between depigmented skin and outer border of hyperpigmented skin
        • This middle layer of hypopigmented skin does not occur in lesions that are progressing more slowly, and is a marker of rapid progression
      • Confettilike depigmentation 2
        • Clustered depigmented macules (1-5 mm in diameter 2) often found at the border of lesions which will rapidly progress
      • Blurred, hypomelanotic borders when lesion is examined under Wood lamp 14

Causes and Risk Factors

Causes

  • Destruction of melanocytes of skin and/or hair follicles 15
    • Pathogenesis unclear, but is attributed to combination of genetics (mostly related to autoimmunity) and environment (oxidative stress has been implicated)

Risk factors and/or associations

Age
  • May occur at any age, but nearly 50% of patients present with disease before age 20 years 2
    • Segmental vitiligo has an earlier age of onset (15.6 years) than vitiligo/nonsegmental vitiligo 16
    • Extended family history of vitiligo is associated with earlier onset of disease 17
  • The following subtypes occur more often in adults than in children: 5
    • Vitiligo universalis
    • Acrofacial vitiligo
    • Mucosal vitiligo
Sex
  • No clear sex predilection overall, but age at peak incidence varies by sex 2
    • Peak incidence for males: fifth decade of life 15
    • Peak incidence for females: first decade of life; therefore, most younger patients are female 15
Genetics
  • Nonmendelian, multifactorial, polygenic pattern with incomplete penetrance 15
    • In white populations, 6% risk for developing vitiligo when a first-degree relative is affected 15
    • 23% risk for developing vitiligo when an identical twin is affected 15
    • Genome-wide studies have identified 50 genetic loci contributing to vitiligo risk, primarily components of innate and adaptive immune systems 2
Ethnicity/race
  • No clear racial predilection 2
Other risk factors/associations
  • Certain phenols are environmental triggers for vitiligo (usually via occupational exposure) 2
    • Monobenzyl ether of hydroquinone
    • 4-tert-butylphenol
    • 4-tert-butylcatechol
    • Rhododendrol
  • Other triggers for vitiligo
    • Imiquimod 2
    • Interferon alfa injections 2
    • Severe sunburn, pregnancy, skin trauma, and emotional stress have been reported to precede onset of lesions 15

Diagnostic Procedures

Primary diagnostic tools

  • Vitiligo is primarily a clinical diagnosis, based on suggestive patient history and physical examination 2
    • In the most common form of vitiligo, physical examination reveals well-circumscribed, symmetrical white macules with distinct borders surrounded by normal skin 5
    • Use Wood lamp examination to confirm diagnosis in fair-skinned patients 2
    • Biopsy is rarely necessary, but can be performed to confirm diagnosis in ambiguous cases 2
  • To aid in treatment selection, diagnostic work-up includes:
    • Assessment of lesion activity (spreading/active versus stable/inactive) based on clinical characteristics
    • Estimate of body surface area affected
  • Consider laboratory testing for comorbid autoimmune thyroid disease at time of diagnosis 15
    • Recommendations vary by expert; a reasonable recommendation is to screen with both TSH and thyroid autoantibodies at diagnosis, then again every 1 to 2 years 7
    • Alternative approach is to reserve testing only for those suggestive of thyroid disease after examination and review of systems 7

Laboratory

  • Thyroid screening
    • Indications:
      • Recommendations vary by expert; a reasonable recommendation is to screen at diagnosis, then repeat every 1 to 2 years 7
        • TSH 7
          • Level may be within reference range, low, or high in autoimmune thyroiditis
        • Thyroid autoantibodies 7
          • Helpful as immune screening marker, especially for those with subclinical autoimmune thyroid disease; may be present in patients who have not yet developed thyroid disease but are at risk for it
            • Thyroid peroxidase antibody (TPO)
            • Thyroglobulin antibody (TGAb)

Other diagnostic tools

  • Wood lamp examination 15
    • Light emitted is 365 nm
    • Depigmented areas fluoresce bright, chalky blue-white
      • Occurs as a result of relatively increased amount of dermal collagen (which fluoresces) owing to decreased or absent melanin
    • Use to accentuate areas of depigmentation, making diagnosis easier in fair-skinned patients
    • Also used to identify blurred, hypomelanotic borders that indicate active, progressive disease

Differential Diagnosis

Most common

  • Differential diagnoses can be grouped by extent of lesions and degree of pigment loss
    • Localized hypopigmentation
      • Tinea versicolor 18
        • Common cutaneous fungal infection (Malassezia furfur) that can cause loss of pigment in persons with darker skin (with fair skin, lesions are often pink); typically affects trunk and shoulders
        • Like vitiligo, lesions present as hypopigmented macules; however, unlike vitiligo they tend to be scaly 6
        • Like vitiligo, lesions fluoresce when exposed to Wood lamp; however, they appear yellow or copper rather than chalky white as in vitiligo
        • Differentiate by identifying typical spores and hyphae via microscopic examination of skin scrapings with potassium hydroxide preparation 6
      • Pityriasis alba 6
        • Skin condition primarily affecting children with atopic dermatitis, which causes dry patches of hypopigmented skin
        • Like vitiligo, hypopigmented lesions may first appear on sun-exposed areas (eg, face); commonly occurs on the cheeks
        • Differentiate by physical examination: lesions of pityriasis alba have diffuse borders that are often ill-defined; unlike vitiligo, may have a fine, powdery scale
      • Nevus anemicus 6
        • Congenital disorder characterized by pale macules or patches of skin
        • Differentiate by diascopy, which involves pressing a glass slide to lesional border; nevus anemicus lesions will be indistinguishable from surrounding skin because they are not truly depigmented
      • Nevus depigmentosus 2
        • Congenital disorder characterized by unilateral hypopigmented macular patch that may be mistaken for segmental, focal, or mixed vitiligo
        • Unlike segmental vitiligo, nevus depigmentosus lesions:
          • Do not expand over time
          • Are typically hypopigmented rather than depigmented
          • Are not associated with leukotrichia
          • Have a jagged, rather than smooth border
        • Wood lamp examination differentiates nevus depigmentosus, which appears off-white, whereas vitiligo is accentuated as bright and chalky white 2
      • Tuberous sclerosis 5
        • Rare genetic disorder that can cause ash-leaf hypopigmented areas on the skin
        • Differentiate by clinical diagnosis: unlike vitiligo, tuberous sclerosis is typically accompanied by seizures, shagreen patches, and angiofibromas
      • Idiopathic guttate hypomelanosis
        • Benign condition common in middle-aged to older adults, characterized by hypo- or depigmented macules on trunk or sun-exposed areas 18
        • May be mistaken for confettilike depigmentation characteristic of active vitiligo 6
        • Guttate hypomelanosis macules are typically small and fixed, whereas vitiligo macules often extend into patches
        • Differentiate by history and clinical appearance. If uncertain, biopsy can aid in diagnosis: melanocytes are present in guttate hypomelanosis 18
    • Localized depigmentation
      • Piebaldism 18
        • Genetic condition characterized by an absence of melanocytes from affected areas of skin, resulting in vitiligolike lesions
        • Physical examination and patient history can distinguish piebaldism from vitiligo 6
          • Piebaldism is congenital and inheritance is autosomal dominant 6
          • Distribution of lesions on forehead (with characteristic patch of white hair frontally), anterior trunk, and shins confirms diagnosis
      • Lichen sclerosus 1
        • Chronic condition that presents as atrophic skin with or without fissures 2
        • Most commonly affects anogenital areas in girls and women and may be mistaken for mucosal vitiligo, although lesions involve skin rather than mucous membrane 1
        • Male genitalia may also be affected
        • A minority of patients have extragenital lesions
        • Differentiate with biopsy 6
          • Loss of interfollicular but not follicular melanocytes
          • Presence of sclerosis
      • Waardenburg syndrome 5
        • Rare genetic disorder characterized by white forelock that may be accompanied by depigmented patches of skin
        • Differentiate by presence of other characteristic symptoms, including hearing loss, neurologic symptoms, and intellectual disability, which are not characteristic of vitiligo
      • Pinta
        • Caused by nonsexual direct transmission of Treponema carateum to skin; endemic in remote tropical regions, especially southern Mexico, Central America, and Caribbean islands
        • Lesions appear and progress through several stages over months to years after exposure
          • Initially, localized erythematous papules appear, usually on arms and legs
          • Later, small scaly, reddish lesions develop at same sites
          • Lesions become more widespread, change color (becoming brown or blue-gray), and ultimately lose pigmentation
        • Diagnose based on history of travel to endemic area, history of characteristic skin changes over time, and identification of treponemal infection (using darkfield examination of skin lesions, VDRL testing, and treponemal antibody absorption test)
    • Widespread hypopigmentation
      • Progressive macular hypomelanosis 6
        • Idiopathic condition associated with species of Propionibacterium causing hypopigmentation in young adults
        • Differentiate from vitiligo by physical examination
          • Unlike vitiligo, lesions are never completely depigmented
          • Lesions have a characteristic distribution, confined to trunk; confluent at midline with macules radiating from margins
      • Hypopigmented mycosis fungoides
        • Variant of T-cell lymphoma that affects younger patients with darker skin; 6 may be mistaken for vitiligo 1
        • Presents with epidermal changes, unlike vitiligo 6
          • Epidermal atrophy appearing as a wrinkled surface
          • Dryness
          • Subtle scaling
        • Differentiation may require multiple biopsies and molecular studies to identify T-cell clonality indicative of lymphoma 1

Treatment

Goals

  • There is no definitive cure for vitiligo
    • For new, actively spreading or progressive lesions:
      • Stabilize depigmentation process 19
    • For any lesion, as desired by patient:
      • Stimulate melanocyte regeneration and achieve repigmentation 20
    • For vitiligo universalis:
      • Create uniform skin tone through chemical depigmentation of remaining pigmented skin, if desired by patient 21

Disposition

Recommendations for specialist referral

  • Refer to a dermatologist for confirmation of diagnosis and discussion of treatment options 18
  • Phototherapy and surgical treatments are performed by or under close supervision of a dermatologist 18
  • Offer all patients psychological support 22
    • Refer children with extensive or visible disease to a psychologist if psychosocial symptoms are identified 5
    • Refer patients and parents of children with vitiligo to support groups for help coping with disease 22
      • Vitiligo Support International 23
      • Vitiligo Research Foundation 24
      • National Vitiligo Foundation, Inc. 25
      • Global Vitiligo Foundation 26

Treatment Options

Consider obtaining quality of life assessment (ie, Dermatology Life Quality Index 27) before treatment and use it to monitor patient satisfaction throughout treatment

For all patients, cosmetic camouflage and avoidance of triggers or aggravating factors (eg, sunburn, skin trauma) are considered first line therapies 3

No further treatment may be warranted if patient is not bothered by symptoms 3

If patient is bothered by symptoms, determine treatment regimen by classification of disease, extent of body surface area affected, and whether lesions are new/actively spreading or established/stable 3

  • Vitiligo/nonsegmental vitiligo (when more than 2%-3% of body surface affected) 3
    • First line therapy 3
      • Narrow band UV-B therapy
        • Gold standard for treatment of adults and children aged 6 years or older 3
        • Use total body treatment for lesions affecting more than 15% to 20% of body; use targeted treatment when lesser areas are involved
        • May combine with potent topical corticosteroids if disease is not extensive
        • Useful for both stabilization and repigmentation
        • Photochemotherapy (psoralen plus UV-A) is considered inferior to narrow band UV-B therapy and not as safe; rarely used 3 28
      • For rapidly progressive disease (or if narrow band UV-B fails to stabilize disease) 3
        • Use mini-pulse systemic corticosteroids (eg, dexamethasone)
          • Often administered 2 consecutive days per week for 3 to 6 months 3
          • Effective in arresting rapid progression in about 90% of patients; limited efficacy for repigmentation 29
    • Second line therapy
      • Use surgical techniques (tissue or cellular grafting) if: 30
        • Disease is stable (no new or growing lesions within 6 months to 2 years)
        • Other attempts at repigmentation have failed
    • Third line therapy
      • Depigmentation therapy (monobenzyl ether of hydroquinone or 4-methoxyphenol) 21
        • Indicated for the following patients: 3
          • Those with vitiligo universalis or other extensive disease
          • Patients must be emotionally stable and willing to adhere to lifelong photoprotection 3
  • Segmental vitiligo (and very limited segmental vitiligo, when less than 2%-3% of body surface affected) 3
    • First line therapy 3
      • Topical calcineurin inhibitors 3
        • May stabilize and stop progression of active disease (may also result in some repigmentation)
        • Preferable to corticosteroid therapy for thin-skinned areas (eg, face, neck) 3
        • Efficacy not clearly demonstrated on other sites 3
        • Do not use for patients younger than 2 years 15
      • Topical corticosteroids
        • May stabilize and stop progression of active disease (may also result in some repigmentation)
        • Preferred choice of therapy for lesions not on face or neck 5
        • Use potent (first choice; ie, betamethasone valerate) or very potent (ie, clobetasol propionate) corticosteroids; dose and frequency of use will depend on several factors including total body surface area affected 3 31
        • Mometasone furoate (which results in negligible adverse effects) is used when systemic absorption is a concern, including for the treatment of: 3
          • Children
          • Thin or large areas of skin
      • Phototherapy 3
        • Targeted narrow band UV-B therapy can be used for both stabilization and repigmentation of disease (excimer laser is especially effective)
        • Combined treatment with calcineurin inhibitors is effective and provides better results than either treatment used alone 3
        • May also be used in combination with topical corticosteroids
    • Second line therapy 9
      • Consider surgical techniques (eg, tissue or cellular grafting) if repigmentation is unsuccessful or unsatisfactory
      • Surgery is reserved for patients with stable, recalcitrant lesions

Drug therapy

  • Calcineurin inhibitors (eg, tacrolimus, pimecrolimus)
    • Off-label use for vitiligo 3
    • For treatment of new and/or active lesions on thin skin (eg, facial or neck lesions) 3
    • Dosing varies; consultation with dermatologist is recommended
  • Corticosteroids
    • Topical corticosteroids
      • Betamethasone valerate 31 0.1% (intermediate potency; first choice) or clobetasol propionate 31 0.05% (very high potency; second choice) 3
        • Use for patients with extrafacial involvement of limited extent
        • Dosing and length of treatment varies; often used in a discontinuous regimen. Consultation with dermatologist is recommended
      • Mometasone furoate topical cream (intermediate potency)
        • Negligible adverse effects compared to other topical steroids; used when systemic absorption is a concern (eg, for children or patients with large affected surface area)
        • Dosing and length of treatment varies; consultation with dermatologist is recommended

Nondrug and supportive care

Protection from sun 5

  • Advised for all patients, particularly for areas of depigmentation
  • Includes sun block, sunscreen (SPF 30 or higher), hats, clothing, and sunglasses

Camouflage (to help with psychological burden of disease) 4

  • Self-tanning lotions
  • Pigmented covering creams
Procedures
Phototherapy

General explanation

  • Narrow band UV-B therapy 3
    • Exposure of affected area to UV-B light with peak wavelength of 311 nm several times per week
    • May be delivered to whole body via an upright booth; table-top delivery systems are also available for treatment of smaller areas
    • Dose is individualized based on skin type, response, and other factors 32
    • Treatment response time is variable; lack of response is usually evident only after 48 exposures 32
    • Excimer laser therapy is another type of narrow band UV-B therapy useful for treating small, localized areas of vitiligo
      • Application of 308 nm UV laser 1 to 3 times per week for an initial course of 12 weeks 4

Indication

  • Narrow band UV-B is the gold standard for treatment of vitiligo/nonsegmental vitiligo affecting more than 2% to 3% of body surface area 3
  • Delivery by excimer laser is reserved for patients with smaller lesions 1
  • Useful for any patient with extensive or rapidly spreading disease 5

Contraindications 33

  • Patients with current:
    • Lupus erythematosus
    • Basal cell nevus syndrome
    • Xeroderma pigmentosum
  • Patients with history of:
    • Melanoma or multiple nonmelanoma skin cancer
    • Arsenic ingestion
    • Radiation exposure

Complications

  • Erythema may occur 12 to 24 hours after treatment, but typically resolves in 24 hours 3
  • Pruritus, desquamation, transient hyperpigmentation, blistering, ulceration, and xerosis may occur 4
  • With laser therapy: erythema and pruritus are possible, but self-limiting 3
Tissue grafting

General explanation 3

  • Grafting of melanocyte-rich tissue from an autologous donor site with normal pigmentation
  • Tissue graft options include:
    • Suction-blister graft
      • Can be performed in outpatient setting and leaves donor site unscathed
      • Not suitable for large lesions and may be time-consuming 3
    • Full-thickness punch graft
      • Easier and less expensive than other options 3
      • Can be performed in outpatient setting and donor site is often unscathed 4
      • Not suitable for large lesions and often does not produce even repigmentation 3
    • Split-thickness skin graft
      • Used less frequently than full-thickness punch, but can cover large areas with a single procedure (up to 200 cm²) 3
      • Requires use of anesthesia 4
      • Harvest sites may be cosmetically displeasing 4

Indication

  • Stable, recalcitrant lesions 4
  • Focal vitiligo 3

Contraindications

  • Disease progression within the last year 5
  • Koebner phenomenon, which can worsen after surgery 4

Complications

  • Suction-blister graft 3
    • Hyperpigmentation at donor site (transient)
  • Full-thickness punch graft
    • Cobblestoned texture of acceptor site 4
    • Scar formation at donor site 3
  • Split-thickness skin graft (complications may be transient or permanent) 9
    • Hypo- or hyperpigmentation
    • Hypertrophic scars at donor site
    • Milia formation at recipient site
    • Contracture
    • Tire pattern appearance
Cellular grafting 3

General explanation

  • Grafting melanocyte-rich cells from autologous donor site with normal pigmentation
  • Cellular grafts 4
    • Can treat larger areas with better cosmetic results when compared with tissue grafts
    • Autologous melanocyte suspension transplant
      • Use of autologous suspension of melanocytes and keratinocytes
      • More complex and time-consuming than tissue grafts
      • Best for patients with focal or segmental vitiligo
    • Noncultured epidermal cellular grafts 3
      • Use of pure cultured melanocytes that were expanded in vitro
      • Can treat lesions up to 500 cm²

Indication

  • Stable, recalcitrant lesions 4
  • Focal or segmental vitiligo 3

Contraindications

  • Disease progression within the last year 5
  • Koebner phenomenon, which can worsen after surgery 4

Complications 3

  • Complications of cellular grafting are rare and transient but may include depigmentation at donor site and inflammatory hyperpigmentation at recipient site

Comorbidities

  • Autoimmune diseases or inflammatory conditions 22 (up to 25% off vitiligo patients 7)
    • Thyroid disease is the most common and well-established comorbid condition 2 (affecting 19% of patients with vitiligo 34)
      • Primary hypothyroidism is most common, followed by Graves disease 7
      • Risk of thyroid disease is high (3- to 8-fold higher than general population); doubles every 5 years 7
    • Less common 7
      • Rheumatoid arthritis
      • Psoriasis
      • Alopecia areata
      • Atopic dermatitis
      • Pernicious anemia
      • Type 1 diabetes mellitus
      • Addison disease
      • Dermatitis herpetiformis
      • Celiac disease
      • Systemic lupus erythematous
      • Sjögren syndrome
      • Myasthenia gravis
      • Inflammatory bowel disease
    • Ocular or otic disease
      • Melanocytes are present in these tissues, so abnormalities associated with vitiligo may cause:
        • Sensorineural hearing loss
          • Common (12%-18%) in both vitiligo/nonsegmental vitiligo and segmental vitiligo 7
          • Majority of patients tested have some acoustic abnormality 35
        • Ocular abnormalities (16%) 7
          • Choroidal anomalies 15
          • Uveitis 15
          • Iritis 15

Monitoring

  • As vitiligo is associated with serious psychiatric morbidities, it is recommended that quality of life be monitored at diagnosis and throughout treatment 15
  • Annually question each patient about onset of thyroid symptoms 7
  • Consider laboratory screening for autoimmune thyroid disease every 1 to 2 years, with or without a positive review of symptoms 7

Complications and Prognosis

Complications

  • Vitiligo patients may suffer from low self-esteem, poor body image, and social isolation 3
    • Prevalence of psychiatric morbidity ranges from 25% to 30% in Western Europe and 56% to 75% in India, where vitiligo is carries a high social stigma 3
  • Hypo- or depigmented areas of skin are vulnerable to sunburn and should always be protected by clothing, hats, or sunscreen

Prognosis

  • Prognosis for vitiligo/nonsegmental vitiligo is variable
  • After the first 1 to 2 years, segmental vitiligo typically stabilizes and does not progress 11
  • New lesions can be triggered by physical and emotional stress
  • Treatment is more challenging for:
    • Acral lesions
    • Older lesions
    • Older patients
  • Relapse is possible at any time and cannot be predicted

Sources

1: Ezzedine K et al: Revised classification/nomenclature of vitiligo and related issues: the Vitiligo Global Issues Consensus Conference. Pigment Cell Melanoma Res. 25(3):E1-13, 2012

Cross Reference

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