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Vulvar lichen sclerosus and vulvar cancer !

Lichen sclerosus is a chronic itchy whitening of vulvar skin. Untreated, it raises the risk of vulvar cancer, a skin cancer of the outer genitals.

Updated 2026-11-01

Structures involved

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Why it happens

What it is

Lichen sclerosus is a chronic inflammatory, likely autoimmune, skin disease of the anogenital area causing white, thin, scarring plaques. Vulvar cancer, most often squamous cell carcinoma, arises through two pathways: one linked to lichen sclerosus in older women and one linked to high-risk HPV in younger women.

Who gets it

Lichen sclerosus is most common in postmenopausal women and also affects prepubertal girls. Vulvar cancer is uncommon, mainly affecting women over 60, although HPV-related cases occur at younger ages. Delayed diagnosis is frequent because itching is often treated as infection.

Causes

  • Lichen sclerosus: autoimmune inflammation, with genetic predisposition and low-oestrogen states
  • HPV-independent vulvar cancer: arises in chronic lichen sclerosus via differentiated vulvar intraepithelial neoplasia (p53 mutation)
  • HPV-associated vulvar cancer: persistent high-risk HPV infection (types 16 and 18) via high-grade squamous intraepithelial lesion
  • Other vulvar cancers: melanoma, Bartholin gland carcinoma, basal cell carcinoma, Paget disease of the vulva

Risk factors

  • Age over 60 and postmenopausal status
  • Untreated or poorly controlled lichen sclerosus
  • Persistent high-risk HPV infection and genital warts
  • Cervical or vaginal intraepithelial neoplasia
  • Smoking
  • Immunosuppression, including HIV infection
  • Other autoimmune diseases (thyroid disease, vitiligo) for lichen sclerosus

Pathophysiology

In lichen sclerosus, T-cell mediated inflammation thins the epidermis and replaces the upper dermis with homogenised collagen, producing fragile white skin, fissures and scarring that can bury the clitoris, fuse the labia minora and narrow the introitus. Chronic inflammation and scratching drive keratinocyte mutations (notably TP53), leading to differentiated VIN and keratinising squamous cell carcinoma. Separately, high-risk HPV oncoproteins E6 and E7 inactivate p53 and Rb, producing high-grade lesions that progress to basaloid or warty carcinoma. Vulvar cancer spreads first to the inguinofemoral lymph nodes.

Classification

  • Lichen sclerosus (benign, chronic, premalignant risk)
  • Differentiated VIN (HPV-independent, high progression risk)
  • Vulvar high-grade squamous intraepithelial lesion (HPV-associated)
  • Invasive vulvar cancer, staged by FIGO system (size, depth, nodes, spread)

Symptoms and signs

  • Intense chronic vulvar itching, often worse at night
  • White, shiny, crinkled plaques, often in a figure-of-eight around vulva and anus
  • Fissures, bruising, pain and dyspareunia
  • Loss of labia minora, buried clitoris and narrowed introitus
  • A lump, ulcer or thickened area that does not heal (suspected cancer)
  • Bleeding or discharge not related to menses

Diagnosis

Clinical
Lichen sclerosus is often diagnosed by its typical appearance; any atypical or persistent lesion needs biopsy. Inspection of the whole anogenital area, cervix and groin.
Tests
Biopsy of the vulva (punch biopsy) to confirm lichen sclerosus when uncertain, and always for suspected cancer or VIN; HPV testing and cervical screening; thyroid tests when autoimmune disease is suspected.
Imaging
For cancer: groin ultrasound, CT, MRI or PET-CT for staging; sentinel lymph node mapping at surgery.

Differential diagnosis

  • Genitourinary syndrome of menopause
  • Lichen planus and lichen simplex chronicus
  • Vulvovaginal candidiasis
  • Vitiligo
  • Contact dermatitis and psoriasis
  • Genital warts and herpes ulcers

Goal of treatment

Control itching and inflammation of lichen sclerosus to prevent scarring and cancer, and treat vulvar cancer with the least mutilating surgery that achieves cure.

Treatment

Medical
Lichen sclerosus: ultrapotent topical corticosteroids are first-line, with a tapering then long-term maintenance schedule; emollients as soap substitutes.
Medical
Second-line for lichen sclerosus: topical calcineurin inhibitors; HPV-related high-grade lesions may be treated with topical imiquimod.
Surgical
Vulvar intraepithelial neoplasia: wide local excision or laser ablation. Invasive cancer: radical local excision or vulvectomy with sentinel node biopsy or inguinofemoral lymphadenectomy.
Medical
Locally advanced cancer: chemoradiation, with surgery in selected cases.
Follow-up
Lifelong follow-up of lichen sclerosus with examination at least yearly and biopsy of any new or persistent lesion; wound care and lymphoedema monitoring after cancer surgery.

Complications

  • Scarring, labial fusion and introital stenosis
  • Sexual dysfunction and urinary difficulty
  • Squamous cell carcinoma of the vulva
  • After cancer surgery: wound breakdown, lymphoedema and altered body image

Prognosis

Lichen sclerosus cannot be cured but is well controlled with topical steroids, which lower the cancer risk. Vulvar cancer detected early, without nodal spread, has a good survival rate; nodal involvement is the main prognostic factor.

Prevention

  • HPV vaccination and smoking cessation
  • Regular self-examination and follow-up for women with lichen sclerosus
  • Prompt biopsy of persistent lesions

Test yourself

Think of the answer, then tap the question to see it.

An older woman has intense vulvar itching and white, shiny, crinkled plaques around the vulva and anus. Diagnosis and first-line treatment?

Lichen sclerosus; ultrapotent topical corticosteroid.

What is the most common histological type of vulvar cancer?

Squamous cell carcinoma.

What are the two pathways to vulvar squamous cell carcinoma?

HPV-associated (younger women, high-grade squamous lesion) and HPV-independent (older women, lichen sclerosus, differentiated VIN with p53 mutation).

To which lymph nodes does vulvar cancer spread first?

The inguinofemoral (groin) lymph nodes.

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Educational information, not medical advice. For any symptom, see a health professional.

Sources: Lichen sclerosus: Causes, Diagnosis, and Images - DermNet (DermNet, 2024); Vulvar Cancer (MedlinePlus (U.S. National Library of Medicine), 2024); Vulvar Cancer Treatment (National Cancer Institute, 2024).

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Representative model, for educational purposes only.

Labia majora highlighted on the Durgical 3D model