Overview
Definition:
Vitiligo is a chronic autoimmune disorder characterized by progressive depigmentation of the skin due to the destruction of melanocytes
It is the most common cause of primary hypopigmentation.
Epidemiology:
Vitiligo affects approximately 0.5% to 2% of the global population, with onset frequently occurring in childhood and adolescence
In pediatric populations, it is estimated to affect 0.1% to 4% of children
There is no significant gender predilection.
Clinical Significance:
Vitiligo in children can have significant psychosocial implications, leading to low self-esteem, social anxiety, and stigmatization
Effective management is crucial not only for repigmentation but also for improving the child's quality of life
Understanding the role of topical calcineurin inhibitors (TCIs) and phototherapy is vital for pediatric residents preparing for DNB and NEET SS examinations.
Clinical Presentation
Symptoms:
Appearance of well-demarcated, depigmented macules or patches
Lesions are typically asymptomatic
Gradual expansion of patches
Predilection for areas exposed to sunlight, mucocutaneous junctions, and sites of trauma (Koebner phenomenon).
Signs:
Milky-white or depigmented macules and patches with sharply defined borders
Variations in size and shape
Possible presence of perifollicular repigmentation in early stages
Absence of scaling or inflammation.
Diagnostic Criteria:
Diagnosis is primarily clinical, based on the characteristic appearance of depigmented lesions
Wood's lamp examination can enhance visualization of depigmented areas by increasing fluorescence
Biopsy is rarely needed unless there is diagnostic uncertainty or suspicion of other conditions.
Diagnostic Approach
History Taking:
Age of onset
Distribution and progression of lesions
Family history of vitiligo or other autoimmune diseases (thyroid disease, diabetes mellitus, pernicious anemia)
History of trauma to affected areas
Associated symptoms suggesting underlying autoimmune conditions.
Physical Examination:
Systematic examination of the entire skin surface to assess the extent and pattern of depigmentation
Note distribution (segmental, generalized, focal, mucocutaneous)
Assess for Koebner phenomenon
Examine nails for leukonychia
Evaluate for signs of associated autoimmune conditions.
Investigations:
Generally not required for diagnosis in typical cases
If associated autoimmune conditions are suspected, baseline investigations may include thyroid function tests (TSH, FT4), fasting blood glucose, and complete blood count with vitamin B12 and folate levels
Skin biopsy is reserved for atypical presentations to rule out other disorders.
Differential Diagnosis:
Tinea versicolor (itchy, scaly, hypopigmented macules, often on trunk)
Post-inflammatory hypopigmentation (history of eczema, psoriasis, or trauma)
Pityriasis alba (mild, ill-defined hypopigmented patches, common in children with atopic dermatitis)
Chemical leukoderma (exposure to phenolic compounds)
Idiopathic guttate hypomelanosis (small, discrete white macules, typically in sun-exposed areas, older adults).
Management
Initial Management:
Education regarding the chronic nature of vitiligo and realistic expectations
Counseling on psychosocial impact and coping mechanisms
Sun protection measures (sunscreen, protective clothing) to prevent sunburn on depigmented areas and potential exacerbation.
Medical Management:
Topical Calcineurin Inhibitors (TCIs): Tacrolimus 0.03% or 0.1% ointment and Pimecrolimus 1% cream are effective, particularly for facial and truncal lesions
They are immunomodulators that can halt disease progression and induce repigmentation by suppressing T-cell mediated destruction of melanocytes
Applied twice daily for 3-6 months or longer
Less associated with skin atrophy than topical corticosteroids
Phototherapy: Narrowband UVB (NB-UVB) is the mainstay for generalized vitiligo, especially in children when applied consistently
Excimer laser (308 nm) is useful for localized, stubborn lesions
Typically administered 2-3 times per week
Response can take several months
Combination therapy (TCIs + phototherapy) often yields better results.
Surgical Management:
Considered for stable vitiligo unresponsive to medical treatment, usually in older children/adolescents
Procedures include skin grafting (split-thickness, full-thickness, melanocyte transplantation) and punch grafting
These are typically performed by experienced dermatologists.
Supportive Care:
Psychological support and counseling for affected children and their families
Peer support groups can be beneficial
Encourage social integration and address stigmatization.
Complications
Early Complications:
Sunburn on depigmented skin
Psychosocial distress
Minimal risk of infection unless skin is traumatized.
Late Complications:
Limited repigmentation despite treatment
Progressive depigmentation
Associated autoimmune diseases may develop or worsen over time
Social isolation and low self-esteem.
Prevention Strategies:
Strict sun protection
Early intervention with effective treatments
Regular psychological assessment and support
Monitoring for associated autoimmune conditions.
Prognosis
Factors Affecting Prognosis:
Early age of onset often portends slower repigmentation and a higher likelihood of progression
Segmental vitiligo generally has a better prognosis than generalized forms
Facial lesions often respond best to treatment
Presence of autoimmune comorbidities may influence prognosis.
Outcomes:
Repigmentation is possible with TCIs and phototherapy, but the degree of success varies significantly
Complete repigmentation is uncommon
Partial repigmentation can be cosmetically significant
Some patients achieve stable disease without progression
TCIs can help prevent further depigmentation in many cases.
Follow Up:
Regular follow-up every 3-6 months is recommended to monitor treatment response, adherence, and assess for new lesions or signs of associated conditions
Duration of treatment can be prolonged (1-2 years or more)
Cessation of treatment should be carefully considered based on response and disease stability.
Key Points
Exam Focus:
TCIs (tacrolimus, pimecrolimus) are immunomodulators useful for facial vitiligo and early disease to halt progression and promote repigmentation
NB-UVB and excimer laser are key phototherapy modalities for more extensive or resistant cases
Koebner phenomenon and autoimmune associations are critical exam points.
Clinical Pearls:
Start TCIs early for facial lesions to prevent significant depigmentation
Combine TCIs with phototherapy for better outcomes in generalized vitiligo
Always counsel parents on realistic expectations and psychosocial impact
Sun protection is paramount.
Common Mistakes:
Over-reliance on topical corticosteroids for long-term vitiligo management, risking atrophy
Delaying phototherapy for widespread disease
Underestimating the psychosocial burden of vitiligo in children
Neglecting to screen for or manage associated autoimmune conditions.