Chronic inflammatory vulvar dermatosis with porcelain-white plaques and architectural change.
Also known as: lichen sclerosus, LS, vulvar lichen sclerosus, kraurosis vulvae
Overview
Chronic, relapsing inflammatory dermatosis predominantly affecting the anogenital skin, characterized by thinning, depigmentation, and architectural destruction. Carries a 4-6% lifetime risk of vulvar squamous cell carcinoma if untreated.
Epidemiology
Bimodal distribution: prepubertal girls and postmenopausal women, with peak incidence in the 6th-7th decade. Female-to-male ratio ~10:1.
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Possible role of chronic occlusion, irritation, and Koebner phenomenon
Pathophysiology
Autoimmune process with T-cell-mediated injury to basal keratinocytes producing characteristic histology of hyperkeratosis, epidermal atrophy, basement membrane thickening, and a homogenous band of hyalinized collagen in the upper dermis with a lichenoid lymphocytic infiltrate beneath.
Clinical presentation
Symptoms
Severe vulvar and perianal pruritus (most common; often worse at night)
Burning, soreness, dyspareunia
Dysuria, urinary symptoms
Dyschezia and constipation if perianal involvement
Children may present with constipation, dysuria, or behavioral changes; itching and visible findings may be missed
Signs / physical exam
Porcelain-white, atrophic, crinkly ('cigarette-paper') plaques on labia minora, clitoral hood, and perianal area in a classic 'figure-of-8' or 'keyhole' distribution sparing the vagina
Architectural change: resorption of labia minora, fusion of clitoral hood, narrowing of introitus
Ecchymoses, fissures, and erosions are common
Lichenification from scratching
Differential diagnosis
Lichen planus — Erosive lesions, oral involvement, Wickham striae, more commonly with painful erosions and dyspareunia
Vitiligo — Symmetric depigmentation without atrophy or architectural change
Atrophic vaginitis — Hypoestrogenic changes, no architectural destruction or porcelain plaques
Vulvar intraepithelial neoplasia (VIN) / SCC — Persistent plaque, ulcer, or mass — biopsy any non-responding or atypical lesion
Sexual abuse (pediatric) — LS can mimic with subepithelial hemorrhage; both require careful evaluation
Diagnostic workup
Diagnostic criteria
Clinical with characteristic appearance is usually sufficient. Punch biopsy is recommended for atypical features, treatment failure, or suspicion of malignancy.
Labs
TSH (associated thyroid autoimmunity)
Glucose if symptomatic
No routine serologies otherwise
Imaging
Not routinely indicated
Diagnostic algorithm
Feature
Lichen Sclerosus
Lichen Planus
Lichen Simplex Chronicus
Typical location
Vulva, perianal (figure-of-8), not vaginal
Vulva, vagina, oral mucosa
Wherever scratched (often labia majora)
Appearance
White, atrophic, 'cigarette-paper' skin
Erosive, Wickham striae, painful
Thick, leathery, lichenified
Architecture
Resorbed labia minora, phimosis
Vaginal adhesions, synechiae
Preserved
First-line tx
Ultrapotent topical steroid (clobetasol)
Ultrapotent topical steroid
Mid-potency steroid + break itch-scratch cycle
Distinguishing vulvar lichen sclerosus from lichen planus and lichen simplex chronicus.
Treatment
First-line
Ultrapotent topical corticosteroid — clobetasol propionate 0.05% ointment is the gold standard
Typical regimen: nightly for 4 weeks, every other night for 4 weeks, twice weekly for 4 weeks, then maintenance 1-2 times weekly indefinitely
Emollients (white petrolatum, zinc oxide) and irritant avoidance (no soaps, fragranced products, tight clothing)
Treatment continues indefinitely — LS is chronic and requires maintenance to prevent flares and malignant transformation
Follow up every 6-12 months for assessment of response and surveillance for malignancy
Second-line / adjunct
Topical calcineurin inhibitors (tacrolimus 0.1% ointment, pimecrolimus 1% cream) — second-line; useful in steroid-resistant or maintenance therapy; FDA black-box warning for theoretical malignancy risk
Intralesional triamcinolone for refractory areas
Topical or systemic retinoids in select cases
Surgery limited to release of agglutination causing functional impairment, treatment of suspected malignancy, or vulvar cancer; surgical excision alone does not cure LS
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