Vitiligo and associated autoimmune disease

01OverviewDefinition, clinical context and the essential points that orientate the chapter.

Vitiligo presents as milk-white macules and patches in which pigment is completely absent. Contrast is often immediately apparent in brown or black skin, while Wood-lamp examination may help detect subtle disease in lighter skin. Common sites include periocular and perioral skin, hands, feet, elbows, knees, genital skin and areas of repeated friction. Koebnerisation means new patches can develop at sites of trauma. Distribution, rate of spread, white hair and previous inflammation help classify the process and estimate treatment response.

Autoimmune destruction of melanocytes is the leading model for non-segmental disease. Autoimmune thyroid disease is the most frequent clinically relevant association; type 1 diabetes, pernicious anaemia, Addison disease and other autoimmune conditions occur less commonly. Screening must combine the recommended thyroid assessment with symptom-led testing rather than ordering an indiscriminate autoimmune panel. The skin itself is physically healthy, but reduced melanin increases sunburn susceptibility and the psychosocial burden can be substantial.

Shared decisions should establish whether the goal is repigmentation, arresting spread, camouflage, depigmentation of very extensive residual pigment, or no active treatment. Site, age, pregnancy potential, disease activity, access and treatment burden matter. Response is assessed over months with consented photographs; absence of early colour change after a few weeks does not prove failure.

Key points

Vitiligo is acquired loss of functioning epidermal melanocytes, producing sharply demarcated depigmented rather than merely pale skin; hair within a patch may also become white.
Non-segmental vitiligo is commonly bilateral and symmetrical around hands, face, body openings and friction sites, whereas segmental disease is usually unilateral and follows a more localised distribution.
Examine in good neutral light and compare with normally pigmented skin; erythema may be less conspicuous in deeply pigmented skin, but complete pigment loss and border definition remain informative.
A Wood lamp can accentuate depigmentation and reveal subtle extent, especially where contrast is low, but it does not replace history, full skin examination or appropriate differential diagnosis.
Ask about thyroid symptoms and personal or family autoimmunity; British dermatology guidance supports thyroid function and antithyroid-antibody screening because autoimmune thyroid disease is an important association.
Treatment choices include camouflage, sun protection, a site-appropriate topical corticosteroid, off-label topical tacrolimus on selected sites, and specialist phototherapy for suitable extensive or progressive disease.
Repigmentation is slow and often begins around follicles; acral areas and patches with white hairs generally respond less reliably than face and trunk.
Discuss psychological, cultural and occupational impact directly. Visible difference can be highly distressing even when affected body-surface area is small.

02AetiologyUnderlying causes, associations and risk factors, with why each one matters.

Autoimmune susceptibility

Non-segmental vitiligo reflects genetic and immune susceptibility in which melanocyte-directed responses cluster with thyroid and other autoimmune diseases.

Oxidative and cellular stress

Melanocyte stress signals may expose antigens and amplify local inflammation, helping explain why friction, sunburn or illness sometimes precedes spread.

Segmental biology

Segmental disease follows a regional unilateral pattern and behaves differently from the usual symmetrical autoimmune phenotype, often stabilising after an earlier active phase.

03PathophysiologyThe causal sequence from the underlying abnormality to symptoms and harm.

1
Melanocytes are depleted
Immune injury reduces functioning epidermal melanocytes, so affected keratinocytes no longer receive melanin and skin appears completely white.
2
Follicles enable recovery
Surviving melanocyte precursors around hair follicles can migrate outward during treatment, producing characteristic islands of perifollicular repigmentation.
3
Trauma recruits activity
Koebnerisation allows friction, scratching, burns or other injury to trigger new lesions in a person with active immune susceptibility.
4
Melanin protection falls
Depigmented skin has reduced ultraviolet protection and burns more readily, although vitiligo itself is not a premalignant lesion.

04Clinical features and red flagsSymptoms, examination findings, patterns of presentation and time-critical warnings.

Complete pigment loss

Well-defined milk-white macules lack normal pigment rather than showing a lighter shade alone; surface scale is absent unless another dermatosis coexists.

Non-segmental pattern

Bilateral patches on acral, facial, peri-orificial and friction sites, often with episodes of spread and stability, support the common autoimmune phenotype.

Segmental pattern

A unilateral regional patch that develops relatively quickly and then stabilises, often in a younger person, suggests segmental vitiligo.

Leucotrichia

White hairs within affected skin indicate follicular pigment loss and predict a lower chance of repigmentation from topical or light treatment.

Inflammatory edge

Rare inflammatory vitiligo has an erythematous or raised margin; scale, marked itch or pain should instead prompt reconsideration of infection or dermatitis.

Autoimmune clues

Neck swelling, temperature intolerance, weight change, palpitations, postural symptoms, polyuria, neuropathy or macrocytic symptoms require targeted endocrine or haematological assessment.

Red flags requiring action

Rapid pigment loss after chemical exposure, new neurological or systemic symptoms, extensive inflammation, ocular symptoms, or features of untreated thyroid or adrenal disease require prompt cause-specific assessment rather than routine cosmetic review.

05InvestigationsWhat to request, why it matters and how to interpret it.

Investigation order

Read from the initial assessment onwards. Tests may run in parallel in urgent care; first-line, preferred, confirmatory, definitive and gold-standard labels appear only when the chapter explicitly states them.

01
Whole-skin and hair examinationFirst step
Why
Confirm extent, distribution, activity clues and follicular pigment reserve.
Interpretation and limitations
Document body sites and white hair without relying on percentage alone; inspect mucosa when symptoms or distribution indicate, and look for a competing scaly or inflammatory process.
02
Wood-lamp examination
Why
Accentuate true depigmentation and identify clinically subtle patches.
Interpretation and limitations
Bright enhancement supports absent epidermal pigment, but fluorescence patterns are not wholly specific and recent topical products can create artefact.
03
Thyroid function and antithyroid antibodies
Why
Detect the most important associated autoimmune disorder.
Interpretation and limitations
Interpret TSH with free thyroid hormones and antibody results in clinical context; a positive antibody without dysfunction predicts risk rather than automatically requiring thyroid treatment.
04
Targeted autoimmune testing
Why
Investigate symptoms suggesting diabetes, Addison disease, pernicious anaemia or another association.
Interpretation and limitations
Choose HbA1c or glucose, full blood count, vitamin B12, morning cortisol or specialist tests from the phenotype; vitiligo alone does not justify every test.
05
Skin biopsy
Why
Resolve atypical inflammatory, infiltrative or treatment-resistant diagnostic uncertainty.
Interpretation and limitations
Biopsy is rarely needed in classic disease; select the active border and provide the pathologist with differential, treatment and site information.

06Differential diagnosisRealistic alternatives and the features that help distinguish them.

Post-inflammatory hypopigmentation

A preceding eczematous or inflammatory eruption, indistinct partial pigment loss and gradual recovery favour post-inflammatory change over complete depigmentation.

Pityriasis versicolor

Fine scale on trunk or proximal limbs and supportive microscopy suggest Malassezia infection, which produces hypo- or hyperpigmentation rather than true white patches.

Pityriasis alba

Ill-defined mildly scaly facial patches in a child with dry or atopic skin retain pigment and usually improve with barrier care.

Chemical leukoderma

Depigmentation matching repeated occupational, cosmetic or household chemical contact may spread beyond the direct site and needs exposure control.

Hypopigmented mycosis fungoides

Persistent variably textured or atrophic patches with unusual distribution or progression warrant biopsy to exclude an uncommon cutaneous lymphoma phenotype.

07ManagementImmediate care, first-line treatment, alternatives and escalation.

01Confirm and classifyEstablish depigmentation and activityFirst stepNew pale patches could represent vitiligo or a mimicking pigment disorder.

1Ask about onset, progression, trauma, preceding rash, chemical exposure, medicines, family history and autoimmune symptoms, then examine the entire skin and hair in neutral light.
2AlternativeUse a Wood lamp when extent or complete pigment loss is uncertain, and sample scale or biopsy only when a specific alternative remains plausible.
3Classify segmental versus non-segmental pattern, record active spread and leucotrichia, and obtain consented baseline photographs using consistent lighting and camera settings.

02Local treatmentBalance repigmentation with site safetyAlternativeThe person wants active treatment for limited vitiligo and no urgent alternative diagnosis is present.

1Discuss camouflage and broad-spectrum sun protection alongside medicine, because contrast, sunburn and visible-light exposure affect daily experience.
2Select a site-appropriate topical corticosteroid for nonfacial skin or discuss off-label tacrolimus for selected facial or flexural areas, with explicit application and review dates.
3Review response and adverse effects after a defined interval, using photographs rather than memory, and refer when progression, sensitive sites or treatment burden exceeds primary-care expertise.

03Extensive or active diseaseEscalate without promising uniform colourEscalationVitiligo is spreading, extensive, psychologically disabling or unresponsive to safe local treatment.

1Refer to dermatology for diagnostic confirmation, disease-activity assessment and discussion of narrowband ultraviolet B phototherapy or other specialist options.
2Explain that treatment commonly takes months, relapse can occur and face or trunk generally repigment more readily than fingers, toes or white-haired skin.
3Assess mental health, work, relationships and access to camouflage or peer support at the same time as physical response, and agree when stopping treatment is reasonable.

04Autoimmune associationScreen and investigate proportionatelyVitiligo is diagnosed or symptoms suggest another autoimmune disorder.

1Arrange thyroid function and antithyroid antibodies in line with British dermatology guidance, documenting any previous thyroid disease or pregnancy context.
2Investigate other autoimmune conditions only from symptoms, examination, family history or abnormal routine results rather than applying an untargeted panel.
3Refer abnormal endocrine results through the appropriate pathway and give urgent advice for collapse, vomiting, severe postural symptoms or hypoglycaemia suggestive of adrenal or diabetic decompensation.

Key medicines and prescribing safety2 treatments · regimens, roles and cautions

Suppresses local immune activity and can promote repigmentation in limited active vitiligo.

Site-appropriate potent topical corticosteroid

Apply a thin layer once daily to selected nonfacial patches for a defined specialist or primary-care course, then review before continuing or stepping down.

Match potency to site and avoid prolonged unsupervised use on face, folds or genital skin; monitor atrophy, telangiectasia, striae, acneiform change and total treated area.

Provides a steroid-sparing local option, particularly where facial or flexural skin makes corticosteroid atrophy a concern.

Tacrolimus 0.1% ointment for selected adult sites

Apply a thin layer twice daily under an explicitly off-label vitiligo plan, with interval review and avoidance of unnecessary ultraviolet exposure.

Vitiligo use is off-label. Explain transient burning, infection precautions and sun protection; avoid use over suspected infection and seek specialist advice in immune compromise, pregnancy or extensive disease.

08ComplicationsImportant consequences, why they occur and why they matter clinically.

Sunburn

Absent melanin lowers natural ultraviolet protection, so exposed patches burn before surrounding skin and require practical photoprotection.

Psychological harm

Stigma, altered identity, anxiety, low mood and social avoidance can be profound, particularly when lesions affect visible or culturally sensitive sites.

Associated autoimmunity

Thyroid dysfunction and less commonly diabetes, adrenal, gastric or other autoimmune disease may add symptoms requiring an independent diagnostic pathway.

Treatment toxicity

Topical corticosteroid atrophy, calcineurin-inhibitor irritation and phototherapy erythema or cumulative burden can outweigh small gains when monitoring is weak.

09Monitoring and follow-upTreatment response, safety checks and longer-term review.

Use consented photographs with stable lighting, distance and body position to track spread and perifollicular repigmentation every few months.
Review topical quantity, exact sites and corticosteroid adverse effects rather than renewing potent treatment indefinitely from an old prescription.
Follow abnormal thyroid results according to thyroid guidance; antibody positivity with normal function requires an agreed reassessment plan rather than automatic levothyroxine.
Reassess mood, avoidance, bullying, work exposure and quality of life because distress does not correlate reliably with body-surface area.
During phototherapy, dermatology records cumulative exposure, erythema, response by site and whether continued treatment remains worthwhile.

10Special situationsVariants, exceptions and circumstances that change the usual approach.

White is different from pale

Vitiligo removes pigment completely; hypopigmentation after eczema or infection usually retains some pigment and often has a history of surface change.

Hair predicts reserve

Repigmentation commonly starts around follicular melanocytes, so leucotrichia signals loss of an important reservoir and a poorer local response.

Contrast changes perception

Summer tanning of unaffected skin can make vitiligo appear to spread even when mapped borders are stable, while sun protection reduces that contrast.

Thyroid antibodies predict risk

Antithyroid antibodies can precede hormone dysfunction; they guide future vigilance but do not by themselves establish a treatment indication.

Stability changes options

Surgical grafting or cellular procedures are restricted to carefully selected stable disease because active immune loss can erase transferred pigment.

11Common pitfallsFrequent interpretation and management errors.

01
Calling every pale patch vitiligo without checking scale, preceding inflammation, sensation and chemical exposure.
02
Using a Wood lamp as a stand-alone diagnosis instead of an adjunct to complete examination.
03
Applying potent corticosteroid continuously to eyelids, face or folds without a review date.
04
Ordering a broad autoimmune panel while omitting the clinically important thyroid assessment.
05
Describing treatment failure before allowing enough time or comparing reliable photographs.
06
Minimising psychosocial impact because vitiligo has no pain or systemic physical impairment.