1. Background information

a. Definition

A chronic inflammatory skin condition affecting mainly the anogenital region, causing itching, pain, and progressive skin changes, with risk of scarring and malignancy.

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b. Clinical classification

By site

• Genital (most common)
  • Vulval (♀)
  • Penile (♂)
• Extragenital (less common)

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By patient group (AKT pattern)

• Women (postmenopausal most common)
• Men (uncircumcised)
• Children (prepubertal)

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c. Etiopathophysiology

• Exact cause unknown

Proposed mechanisms:

• Autoimmune (association with thyroid disease, vitiligo)
• Genetic predisposition
• Chronic irritation / trauma

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Pathological effects

• Inflammation → epidermal thinning + dermal sclerosis
• → fragile, white “parchment-like” skin
• → scarring and architectural changes

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Complication (AKT critical)

• ↑ risk of Vulval squamous cell carcinoma (~5%)

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2. Assessment

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a. Clinical presentation

Symptoms

• Intense pruritus (key feature)
• Soreness / pain
• Dyspareunia
• Dysuria (if severe)

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Signs (AKT classic)

• Porcelain-white plaques
• Thin, shiny “parchment-like” skin
• Ecchymosis / fissures
• Scarring

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Female features

• “Figure-of-8” pattern around vulva and anus
• Loss of labial architecture
• Introital narrowing

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Male features

• Tight foreskin (phimosis)
• White patches on glans

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Red flags

• Persistent ulceration
• Induration / lump
• Non-healing lesions

→ think malignancy

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b. Relevant investigations

NICE CKS approach: clinical diagnosis

• Usually no biopsy required if classic

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Indications for biopsy:

• Diagnostic uncertainty
• Suspicion of malignancy
• Treatment-resistant disease

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Additional

• Consider autoimmune screen if indicated

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AKT pearl

• Do not delay treatment waiting for biopsy if classic

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3. Management (UK NICE CKS-based)

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a. Emergency care

• Not usually required

Rare scenarios:

• Severe urinary obstruction (e.g. phimosis) → urgent urology

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b. Referral

Refer urgently (2WW)

• Suspicion of cancer (non-healing ulcer, mass)

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Routine referral

• Diagnostic uncertainty
• Poor response to treatment
• Children (often specialist-led)
• Severe scarring / anatomical changes

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c. Primary care management

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1. First-line treatment (AKT MUST KNOW)

• Ultra-potent topical corticosteroid
  • e.g. clobetasol propionate

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Typical regimen (AKT pattern)

• Daily for 1 month
• Alternate days for 1 month
• Twice weekly for 1 month
  → then maintenance

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2. Maintenance therapy

• Intermittent steroid use (e.g. 1–2× weekly)
• Prevent relapse

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3. General measures

• Emollients (soap substitute)
• Avoid irritants (perfumed products)
• Good genital hygiene

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4. Male management

• Consider circumcision if:
  • Persistent phimosis
  • Poor response to steroids

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5. Follow-up (AKT important)

• Long-term follow-up required
• Educate patient on:
  • Self-examination
  • Cancer risk

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AKT Exam Pearls

• Intense vulval itching + white plaques = lichen sclerosus
• First-line = ultra-potent topical steroid (clobetasol)
• “Figure-of-8” distribution (♀) = classic
• Risk of squamous cell carcinoma → long-term follow-up
• Biopsy if:
  • Atypical
  • Not responding
  • Suspicious lesion