Dermatology · PANCE / PANRE

Vitiligo

Acquired autoimmune depigmenting disorder with discrete milky-white macules and patches due to melanocyte loss.

Also known as: vitiligo, leukoderma, depigmentation

Overview

An acquired chronic depigmenting disorder of the skin and mucous membranes characterized by well-demarcated milky-white macules and patches resulting from progressive loss of functional epidermal melanocytes.

Epidemiology

Worldwide prevalence ~0.5-2%. Onset before age 20 in 50%. No sex or racial predilection, but psychosocial impact greater in skin of color. Family history in ~20%.

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Question 1DermatologyMedium
A 28-year-old male has a 4-year history of white patches on his bilateral dorsal hands and face that have expanded slowly over time. Vitiligo is confirmed on Wood lamp examination. He is distressed about his appearance. Which of the following is the most appropriate initial therapy?
  • ANarrowband UVB phototherapy
  • BTopical tacrolimus
  • CSystemic corticosteroids
  • DExcimer laser
Reveal answer & full explanation
Correct answer: B — Topical tacrolimus
  • ANarrowband UVB phototherapy
  • BTopical tacrolimus
  • CSystemic corticosteroids
  • DExcimer laser

Why Topical tacrolimus is correct

  • Vitiligo is caused by autoimmune destruction of melanocytes
  • For limited vitiligo (under 10% body surface area (BSA)), first-line options are topical corticosteroids or topical calcineurin inhibitors (tacrolimus, pimecrolimus)
  • Topical calcineurin inhibitors such as tacrolimus are preferred for the face and sensitive areas given their favorable side-effect profile compared to topical corticosteroids
  • This patient has bilateral dorsal hands and face involvement; the face component makes tacrolimus the preferred initial choice

Why the others are wrong

  • A) Narrowband UVB phototherapy — narrowband ultraviolet B (NB-UVB) phototherapy (311-313 nm) is most effective for widespread (extensive) vitiligo, stimulating melanocyte migration and proliferation; it is not the initial therapy for limited disease
  • C) Systemic corticosteroids — systemic corticosteroids carry significant side-effect risk and are not standard first-line therapy for limited vitiligo
  • D) Excimer laser — excimer laser is a targeted phototherapy option, generally used when topical agents and field phototherapy have been tried

Additional high-yield points

  • Extensive vitiligo: narrowband UVB phototherapy (311-313 nm)
  • Ruxolitinib cream 1.5%: first FDA-approved topical Janus kinase 1/2 (JAK1/2) inhibitor for non-segmental vitiligo (approved 2022)
  • Afamelanotide implant plus NB-UVB: used for darker skin types
  • Cosmetic options: camouflage makeup, self-tanners
  • Realistic expectations: repigmentation takes 3-12 months; face and neck respond best; hands and feet respond poorly
Question 2DermatologyMedium
A 31-year-old woman presents 8 weeks postpartum for evaluation of new patchy skin depigmentation involving the dorsa of her hands, periorbital skin, and perioral area. The patches are well-demarcated, asymptomatic, and milky-white; Wood lamp examination accentuates the depigmentation. She is not breastfeeding and uses condoms for contraception. She has no other medical problems. Which of the following is the most appropriate initial treatment?
  • ATopical tacrolimus
  • BNarrowband UVB phototherapy
  • COral methotrexate
  • DSystemic corticosteroids
Reveal answer & full explanation
Correct answer: A — Topical tacrolimus
  • ATopical tacrolimus
  • BNarrowband UVB phototherapy
  • COral methotrexate
  • DSystemic corticosteroids

Why Topical tacrolimus is correct

  • This patient has nonsegmental vitiligo involving the face and acral surfaces — an autoimmune destruction of melanocytes that can be triggered or exacerbated by the postpartum immunologic shift
  • For limited disease, especially on the face and other sensitive sites, topical calcineurin inhibitors (tacrolimus, pimecrolimus) are first-line
  • They are preferred over potent topical steroids because they avoid skin atrophy and telangiectasias with prolonged use
  • Topical tacrolimus is safe, effective, and well-tolerated for facial and limited vitiligo
  • Because she is not breastfeeding, the usual lactation cautions around systemic agents do not apply, but initial therapy is still topical for limited disease

Why the others are wrong

  • Narrowband UVB phototherapy — an excellent option for widespread or treatment-resistant vitiligo but not initial therapy for a few facial/acral patches, and logistically demanding (2-3 sessions/week) (right-concept-wrong-setting)
  • Oral methotrexate — reserved for rapidly progressive or extensive vitiligo refractory to first-line therapy; disproportionate for limited disease (overtreatment)
  • Systemic corticosteroids — sometimes used as oral mini-pulse therapy for rapidly progressive disease, but the adverse-effect profile makes them inappropriate as initial treatment of limited stable vitiligo (overtreatment)

Additional high-yield points

  • Face and neck vitiligo respond best to treatment; acral and lip/tip areas respond least — set expectations early
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Risk factors

  • Polygenic inheritance (NLRP1, PTPN22, TYR, HLA, FOXP3 loci)
  • Personal or family history of autoimmune disease: thyroid (most common — Hashimoto, Graves), pernicious anemia, Addison disease, type 1 diabetes, alopecia areata, rheumatoid arthritis
  • Koebner phenomenon — trauma, friction, sunburn at sites of skin injury
  • Stress (anecdotal)
  • Chemical exposure (phenol derivatives, monobenzone — chemical leukoderma)

Pathophysiology

Autoimmune destruction of melanocytes by CD8+ cytotoxic T-cells targeting melanocyte antigens (tyrosinase, MART-1, gp100). Interferon-γ-driven CXCL9/CXCL10 chemokines recruit autoreactive T-cells. Oxidative stress (impaired antioxidant defenses in melanocytes) and intrinsic melanocyte fragility contribute. JAK-STAT pathway central — basis for new topical JAK inhibitor therapy.

Clinical presentation

Symptoms

  • Most often asymptomatic; mild pruritus at active edges in some patients
  • Psychosocial distress, depression, anxiety, decreased quality of life — especially in skin of color and visible areas

Signs / physical exam

  • Well-demarcated milky-white (chalk-white) macules and patches — completely depigmented (vs hypopigmented)
  • Distribution patterns:
  • • Non-segmental (most common, 85-90%): symmetric, on periorificial face (around eyes, mouth), dorsal hands, axillae, genitalia, elbows, knees; progressive course
  • • Segmental: unilateral, dermatomal or quasi-dermatomal distribution; younger patients; rapid early progression then stable
  • • Acrofacial: face, dorsal hands and feet; common variant
  • • Universal: >80% BSA depigmentation
  • Wood's lamp examination: bright milky-white fluorescence highlights subclinical lesions
  • Leukotrichia (white hairs) within patches — poor prognostic sign for repigmentation
  • Confetti-like depigmentation = active rapid progression

Classic findings

Symmetric, well-demarcated, completely depigmented macules around eyes, mouth, hands, and genitalia — accentuated under Wood's lamp.

Differential diagnosis

  • Tinea versicolor — Hypopigmented finely scaling macules on trunk; KOH 'spaghetti and meatballs'; Wood's lamp pale yellow
  • Pityriasis alba — Ill-defined hypopigmented patches on face of children with atopy; mild scale; partial pigment loss
  • Post-inflammatory hypopigmentation — History of preceding dermatitis/inflammation; not completely depigmented
  • Tuberous sclerosis — Ash-leaf macules present at birth; epilepsy, intellectual disability, facial angiofibromas, shagreen patch
  • Halo nevus — Pigmented nevus with surrounding depigmented ring; benign
  • Idiopathic guttate hypomelanosis — Multiple small (2-5 mm) hypopigmented macules on sun-exposed extremities in older adults
  • Lichen sclerosus — Porcelain-white atrophic plaques, especially genital and figure-of-eight perianal
  • Hypopigmented mycosis fungoides — Adult-onset hypopigmented patches refractory to therapy; biopsy

Diagnostic workup

Diagnostic criteria

Clinical: characteristic acquired depigmented macules ± Wood's lamp confirmation.

Labs

  • Clinical diagnosis — Wood's lamp confirms depigmentation
  • TSH and TPO antibodies (screen autoimmune thyroid disease) at diagnosis and periodically (every 1-2 years)
  • Consider CBC, B12, vitamin D, ANA based on symptoms/risk
  • Skin biopsy rarely needed: absence of melanocytes on H&E and S-100/MART-1 stains

Imaging

  • Not indicated

Diagnostic algorithm

PatternDistributionCourse / Therapy Notes
Non-segmental (85-90%)Symmetric — periorificial, hands, axillae, genitaliaProgressive; NBUVB + topical ruxolitinib / TCI / steroid
SegmentalUnilateral, dermatomalYounger; early rapid then stable; good surgical candidate
AcrofacialFace + dorsal hands/feetCommon variant; similar to non-segmental
Universal>80% BSA depigmentedConsider depigmentation therapy (MBEH)
MucosalLips, genitaliaOften refractory to topicals; JAK inhibitor, NBUVB targeted
Vitiligo clinical patterns and management considerations.

Treatment

First-line

  • Topical corticosteroids: high-potency (clobetasol 0.05%, betamethasone dipropionate 0.05%) BID × 2-4 months for limited disease — pulse therapy (e.g., 2 weeks on / 2 weeks off) to limit atrophy
  • Topical calcineurin inhibitors: tacrolimus 0.1% ointment BID — preferred for face/intertriginous (no atrophy); equally effective as topical steroids in many trials
  • Topical JAK inhibitor: ruxolitinib 1.5% cream BID (FDA-approved 2022 for nonsegmental vitiligo ages ≥12) — significant repigmentation in 50% by 6-12 months
  • Narrowband UVB phototherapy (311-313 nm) 2-3x/week — first-line for widespread disease; combine with topicals for additive effect
  • Excimer laser (308 nm) for localized lesions
  • Sunscreen daily — protect depigmented areas (no melanin protection) and reduce contrast with surrounding skin
  • Cosmetic camouflage: self-tanners (dihydroxyacetone), tinted concealers, micropigmentation

Rapidly progressive / active vitiligo

  • Short courses of oral minipulse corticosteroids (dexamethasone 2.5 mg on 2 consecutive days/week × 3-6 months) to halt progression
  • Combine with NBUVB phototherapy

Stable segmental or localized

  • Surgical melanocyte transplantation: punch grafting, suction blister grafting, melanocyte-keratinocyte transplantation
  • Best results in stable disease >1 year, segmental disease, and areas with intact hair pigment

Extensive (>50% BSA)

  • Depigmentation therapy: monobenzyl ether of hydroquinone (MBEH) 20% cream — permanent depigmentation of remaining pigmented skin for cosmetic uniformity
  • Irreversible — counsel extensively

Second-line / adjunct

  • Afamelanotide (α-MSH analog) + NBUVB — promising in trials
  • Oral JAK inhibitors (ritlecitinib, upadacitinib) — emerging evidence
  • Antioxidants (ginkgo biloba, vitamin E, polypodium leucotomos) — limited evidence
  • Mental health support, vitiligo support groups; address quality of life as actively as the skin disease

Complications

  • Sunburn and increased risk of skin cancer in depigmented areas (though epidemiologic studies surprisingly show LOWER skin cancer rates in vitiligo patients, possibly from increased sun-protective behavior and altered immunity)
  • Psychosocial: depression, anxiety, social isolation, low self-esteem, sexual dysfunction
  • Associated autoimmune diseases — particularly autoimmune thyroid disease
  • Ocular: uveitis, iritis, retinal pigmentary abnormalities (Vogt-Koyanagi-Harada syndrome)
  • Hearing: sensorineural hearing loss (rare; melanocyte loss in inner ear)

PANCE pearls

  • Wood's lamp examination is the bedside test — depigmented vitiligo glows milky-white; hypopigmented conditions (versicolor, post-inflammatory) do not.
  • Screen all vitiligo patients for autoimmune thyroid disease at diagnosis and periodically.
  • Topical ruxolitinib (JAK inhibitor) is now first-line for nonsegmental vitiligo — significant cultural shift in vitiligo therapy since 2022 FDA approval.
  • Leukotrichia (white hairs in patch) predicts poor repigmentation response because hair follicle melanocyte reservoir is depleted.
  • Vitiligo is not 'just cosmetic' — its psychosocial impact often exceeds objective severity; address mental health proactively.

References

  • AAD 2023 — Joint AAD-NPF-Vitiligo Working Group Updated Recommendations for the Management of Vitiligo (Rosmarin et al., J Am Acad Dermatol)
  • VGICC 2023 — Vitiligo Global Issues Consensus Conference Guidelines (Taïeb et al., Br J Dermatol; updated)
  • FDA Ruxolitinib — FDA Approval of Topical Ruxolitinib 1.5% Cream for Nonsegmental Vitiligo (2022)

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