Overview
Vitiligo
1. Clinical Overview
Summary
Vitiligo is a Chronic Acquired Pigmentary Disorder characterised by Loss of Melanocytes resulting in Well-Demarcated White (Depigmented) Macules and Patches on the skin. It affects approximately 0.5-2% of the population worldwide, with onset typically in Childhood or Young Adulthood. The condition is believed to be Autoimmune in pathogenesis, involving T-Cell-Mediated Destruction of Melanocytes. Vitiligo is classified as Segmental (Dermatomal distribution, Stable, Less associated with autoimmunity) or Non-Segmental (Generalised) (Symmetrical, Progressive, Associated with autoimmune conditions like Thyroid disease). Vitiligo has significant Psychosocial Impact, particularly in darker-skinned individuals where contrast is more visible. Treatment aims to Halt Progression and Repigment using Topical Corticosteroids or Calcineurin Inhibitors, Phototherapy (Narrowband UVB), And Surgical Grafting for stable, limited disease. [1,2,3]
Clinical Pearls
"Well-Demarcated White Patches, Otherwise Normal Skin": Classic appearance. No scaling.
"Screen for Thyroid Disease": Especially in Non-Segmental Vitiligo. Check TFTs and Thyroid Antibodies.
"Koebnerisation": New lesions can appear at sites of trauma.
"Narrowband UVB is Mainstay": For widespread or progressive disease.
2. Epidemiology
Demographics
| Factor | Notes |
|---|---|
| Prevalence | ~0.5-2% worldwide. |
| Age of Onset | ~50% before age 20. Peak 10-30 years. |
| Sex | Equal. Females seek treatment more often. |
| Ethnicity | All ethnicities. More noticeable in darker skin. |
| Family History | ~20-30% have affected first-degree relative. |
Associated Conditions
| Condition | Notes |
|---|---|
| Autoimmune Thyroid Disease | Hashimoto's thyroiditis, Graves' disease. Most common association (~15-25%). |
| Type 1 Diabetes Mellitus | |
| Pernicious Anaemia | |
| Addison's Disease | |
| Alopecia Areata | |
| Rheumatoid Arthritis | |
| Psoriasis |
3. Pathophysiology
Mechanism (Autoimmune Hypothesis – Primary)
- Genetic Susceptibility: HLA associations (HLA-A2, HLA-DR4). Other risk genes (NLRP1, TYR, PTPN22).
- Environmental Trigger: Stress, Trauma (Koebner), Chemicals (Phenols), Sunburn.
- Oxidative Stress: Melanocytes susceptible to oxidative damage. Accumulation of H₂O₂.
- Autoimmune Attack: Autoreactive Cytotoxic T Cells (CD8+) target and destroy melanocytes.
- Melanocyte Destruction: Loss of melanin production → Depigmentation.
Other Theories
- Neural Theory: Neuropeptides damage melanocytes (More relevant to Segmental Vitiligo).
- Biochemical/Oxidative Stress: Defective antioxidant mechanisms in melanocytes.
4. Classification
Types
| Type | Features | Associated Autoimmunity |
|---|---|---|
| Non-Segmental (Generalised) | Most common (~85-90%). Symmetrical distribution. Progressive. Often acrofacial initially. Multiple patches. | Yes (Thyroid, T1DM, etc.) |
| Segmental | Dermatomal or quasi-dermatomal. Unilateral. Rapid onset, Then stable. Often overlying white hair (Leukotrichia). Childhood onset. | Less common |
| Mixed | Features of both. | |
| Focal | One or few patches in one area. May evolve. | |
| Mucosal | Only mucous membranes. | |
| Universal | >80% body surface depigmented. |
5. Clinical Presentation
Skin Lesions
| Feature | Notes |
|---|---|
| Macules / Patches | Well-demarcated. Chalk-white (Depigmented). Round or irregular. |
| Surface | Normal skin texture. No scaling. |
| Size | Variable. Millimetres to large patches. |
| Number | Single to multiple. |
| Distribution (Non-Segmental) | Symmetrical. Acrofacial prominent (Face, Lips, Hands, Feet). Periocular, Periorificial. Body folds, Genitalia. Extensor surfaces. |
| Distribution (Segmental) | Unilateral. Follows or quasi-follows dermatome. |
| Leukotrichia | White hair within patch (Follicular depigmentation). More common in Segmental. |
| Koebner Phenomenon | Lesions at sites of trauma (Cuts, Friction). |
Symptoms
Examination
Usually Asymptomatic. Rarely pruritus.
Common presentation.
Significant Psychosocial Impact
Cosmetic disfigurement, Stigma (Particularly darker skin tones), Anxiety, Depression, Reduced quality of life.
6. Differential Diagnosis
| Condition | Key Features |
|---|---|
| Pityriasis Versicolor | Hypo- (Or Hyper-) pigmented macules. Fine scale. KOH positive (Yeast). |
| Pityriasis Alba | Ill-defined hypopigmented patches. Fine scale. Children. Face common. Associated with atopy. |
| Post-Inflammatory Hypopigmentation | Following inflammation (Eczema, Psoriasis). History of prior lesion. |
| Tinea | Annular. Scaly border. KOH positive. |
| Leprosy | Hypopigmented anaesthetic patches. Endemic areas. |
| Chemical Leukoderma | Exposure to phenolic compounds. Occupational. |
| Piebaldism | Congenital. White forelock. Stable. |
| Tuberous Sclerosis (Ash Leaf Macules) | Congenital. Oval or Ash-leaf shaped. Associated features. |
| Idiopathic Guttate Hypomelanosis | Tiny (2-5mm) well-defined white macules. Shins, Forearms. Older adults. |
7. Investigations
Diagnosis is Clinical
- Based on History and Examination.
- Wood's Lamp enhances lesions.
Screen for Associated Autoimmune Conditions
| Test | Notes |
|---|---|
| Thyroid Function Tests (TFTs) | TSH, Free T4. |
| Thyroid Antibodies | Anti-TPO, Anti-Thyroglobulin. |
| Fasting Glucose / HbA1c | If Diabetes suspected. |
| FBC, Vitamin B12, Intrinsic Factor Abs | If Pernicious anaemia suspected. |
| Cortisol / ACTH | If Addison's suspected (Rare). |
Skin Biopsy
- Usually NOT required.
- May be done if diagnosis uncertain.
- Shows Absence of Melanocytes and Absent Melanin in epidermis.
8. Management
Management Algorithm
VITILIGO DIAGNOSED
(Clinical + Wood's Lamp)
↓
SCREEN FOR AUTOIMMUNE CONDITIONS
- TFTs, Thyroid Antibodies
- Annually if negative initially
↓
CLASSIFY
- Segmental vs Non-Segmental
- Extent (% BSA)
- Stability (Active/Progressive vs Stable)
↓
GENERAL MEASURES (ALL)
┌──────────────────────────────────────────────────────────┐
│ **SUN PROTECTION** │
│ - Vitiliginous skin burns easily (No melanin) │
│ - High SPF sunscreen │
│ - Sun avoidance │
│ │
│ **CAMOUFLAGE** │
│ - Cosmetic cover-up creams (Matching skin tones) │
│ - Self-tanning lotions (DHA-based) │
│ │
│ **PSYCHOLOGICAL SUPPORT** │
│ - Significant psychosocial impact │
│ - Counselling, Support groups │
└──────────────────────────────────────────────────────────┘
↓
MEDICAL TREATMENT
┌──────────────────────────────────────────────────────────┐
│ **LIMITED DISEASE (less than 10-20% BSA)** │
│ │
│ **FIRST-LINE: TOPICAL THERAPY** │
│ - **Potent Topical Corticosteroids** (Face/Flexures: │
│ Moderate potency e.g., Mometasone. Body: Potent e.g., │
│ Betamethasone). Use pulsed regimen (Alternating days │
│ or weekends) to reduce atrophy. │
│ - **Topical Calcineurin Inhibitors** (Tacrolimus 0.1%, │
│ Pimecrolimus). Preferred for **Face and Flexures** │
│ (Less atrophy risk). Can be used long-term. │
│ - Duration: Trial for 3-6 months. Assess response. │
│ │
│ **SECOND-LINE** │
│ - **Narrowband UVB Phototherapy** (For limited areas │
│ if topicals fail, Or combine with topicals) │
└──────────────────────────────────────────────────────────┘
↓
WIDESPREAD / PROGRESSIVE DISEASE
┌──────────────────────────────────────────────────────────┐
│ **FIRST-LINE: PHOTOTHERAPY** │
│ - **Narrowband UVB (NB-UVB)** (311-313nm) │
│ - 2-3 times per week │
│ - Duration: 6-12 months (Or longer) │
│ - Best results on face, Neck, Trunk │
│ - Can combine with topical steroids/Tacrolimus │
│ - Home phototherapy units for long-term │
│ │
│ **SYSTEMIC THERAPY (Rapidly Progressive)** │
│ - **Oral Mini-Pulse Corticosteroids** │
│ (e.g., Dexamethasone 2.5-5mg on 2 consecutive days/ │
│ week for 3-6 months). Limits progression. │
│ - **Methotrexate, Azathioprine** (Rarely, For severe │
│ progressive) │
│ │
│ **JAK INHIBITORS (Emerging)** │
│ - **Ruxolitinib 1.5% Cream** (Opzelura) – FDA approved │
│ for non-segmental vitiligo. Topical JAK1/JAK2 │
│ inhibitor. Promotes repigmentation. │
└──────────────────────────────────────────────────────────┘
↓
STABLE, LOCALISED, TREATMENT-RESISTANT
┌──────────────────────────────────────────────────────────┐
│ **SURGICAL TREATMENT** │
│ - For **Stable disease (>1-2 years)** not responding │
│ to medical therapy │
│ - **Punch Grafting**: Small plugs from normal skin │
│ transplanted to depigmented areas. │
│ - **Suction Blister Epidermal Grafting**: Epidermal │
│ sheet from blister. │
│ - **Melanocyte Transfer / Culture**: Advanced. │
│ - Best for Segmental Vitiligo. │
│ │
│ **DEPIGMENTATION (Universal Vitiligo)** │
│ - Monobenzone cream. Depigments remaining normal skin │
│ to achieve uniform appearance. │
│ - Irreversible. Major decision. │
└──────────────────────────────────────────────────────────┘
9. Complications
| Complication | Notes |
|---|---|
| Sunburn | Depigmented skin highly susceptible. Increased skin cancer risk. |
| Psychosocial Impact | Stigma, Low self-esteem, Anxiety, Depression. |
| Associated Autoimmune Diseases | Monitor for thyroid disease especially. |
| Ocular Abnormalities | Iritis, Uveitis (Rare). Consider ophthalmology if symptoms. |
| Hearing Loss | Melanocytes in inner ear. Very rare. |
10. Prognosis and Outcomes
| Factor | Notes |
|---|---|
| Course | Unpredictable. May progress, Stabilise, Or spontaneously repigment partially (~10-20%). |
| Repigmentation | Best on face, Neck. Worst on acral areas (Hands, Feet), Bony prominences, Mucosal. Perifollicular repigmentation (From hair follicle melanocytes). |
| Treatment Response | Variable. NB-UVB and Topicals can achieve significant repigmentation in many. Requires months of treatment. |
| Segmental Vitiligo | Often stabilises early. Good candidate for surgery. |
11. Evidence and Guidelines
Key Guidelines
| Guideline | Organisation | Key Recommendations |
|---|---|---|
| Vitiligo | BAD, EADV | Topical steroids/Calcineurin inhibitors first-line for limited. NB-UVB for widespread. Screen for thyroid. Psychological support. |
12. Patient and Layperson Explanation
What is Vitiligo?
Vitiligo is a condition where patches of skin lose their colour and become white. This happens because the cells that produce skin pigment (Melanocytes) are destroyed, Likely by the body's own immune system.
What are the symptoms?
- White patches on the skin. Often symmetrical.
- Common areas: Face (Around eyes and mouth), Hands, Feet, Arms, Body folds.
- Usually no other symptoms (Not itchy or painful).
What causes it?
The exact cause is unknown, But it is believed to be an autoimmune condition. It can run in families and may be triggered by stress, Sunburn, Or skin injury.
Is it contagious?
No. Vitiligo is NOT contagious and cannot be spread from person to person.
How is it treated?
Treatment aims to restore colour:
- Creams: Steroid or tacrolimus creams (Best for face).
- Light Therapy (Phototherapy): UV light to stimulate repigmentation.
- Surgery: Skin grafting for stable patches.
- Camouflage: Cover-up creams.
- Sun Protection: To prevent burning of white skin.
What is the outlook?
Vitiligo is a chronic condition. Patches may spread, Stay stable, Or sometimes regain colour naturally. Treatment can help repigment, But it often takes months and results vary.
13. References
Primary Sources
- Ezzedine K, et al. Vitiligo. Lancet. 2015;386(9988):74-84. PMID: 25596811.
- Taieb A, et al. Guidelines for the management of vitiligo (EADV Task Force). Br J Dermatol. 2013;168(1):5-19.
- Rodrigues M, et al. New discoveries in the pathogenesis and classification of vitiligo. J Am Acad Dermatol. 2017;77(1):1-13.
14. Examination Focus
Common Exam Questions
- Classic Appearance: "Describe the typical skin lesion in vitiligo."
- Answer: Well-demarcated, Chalk-white (Depigmented), Macules or Patches with Normal skin texture (No scaling).
- Associated Condition: "What autoimmune condition is most commonly associated with Vitiligo?"
- Answer: Autoimmune Thyroid Disease (Hashimoto's, Graves').
- First-Line Treatment (Limited): "What is the first-line topical treatment for vitiligo on the face?"
- Answer: Topical Calcineurin Inhibitors (Tacrolimus 0.1% or Pimecrolimus) (Preferred due to lower risk of atrophy) OR Moderate-potency Topical Corticosteroids.
- Phototherapy: "What type of phototherapy is used for widespread vitiligo?"
- Answer: Narrowband UVB (NB-UVB) (311-313nm).
Viva Points
- Segmental vs Non-Segmental: Segmental is unilateral, Stable, Less autoimmune association.
- Koebner Phenomenon: New lesions at trauma sites.
- Wood's Lamp: Enhances visualisation (Especially in fair skin).
- Perifollicular Repigmentation: From hair follicle melanocytes.
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