Vulvar lichen sclerosus
Vulvar lichen sclerosus is a chronic inflammatory dermatosis causing porcelain-white atrophic plaques, itching, and scarring of the vulva, confirmed by biopsy and treated with an ultrapotent topical corticosteroid to relieve symptoms and reduce the lifelong risk of vulvar squamous cell carcinoma.
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What this article covers
- Overview
Chronic inflammatory dermatosis of the anogenital skin: vulvar lichen sclerosus is a chronic, progressive inflammatory condition of unknown etiology, driven by inflammation and abnormal fibroblast function that leads to dermal fibrosis.
- Epidemiology
Bimodal peaks: most common in prepubertal girls and in women in the peri- and postmenopausal years, with a smaller peak before puberty.
- Risk factors
Postmenopausal or prepubertal hormonal state: estrogen-deficient states (postmenopause) and prepuberty are the two settings in which the disease clusters.
- Pathophysiology
Chronic inflammation triggers fibroblast dysfunction: an immune-mediated inflammatory infiltrate in the dermis leads to abnormal fibroblast activity.
- Clinical features
Symptom triad, worse at night: vulvar pruritus, burning, and dyspareunia, classically worse at night, are the presenting complaint; associated dysuria and dyschezia occur when perianal or periurethral skin is involved.
- Differential diagnosis
Vulvar squamous cell carcinoma: presents with leukoplakia, an exophytic mass, ulceration, or a lesion that bleeds with manipulation, usually in a postmenopausal patient; a discrete invasive tumor on…
- Diagnosis
Vulvar punch biopsy confirms the diagnosis: the diagnosis is established clinically from the characteristic porcelain-white, atrophic, figure-eight plaques, but biopsy should be performed to confirm…
- Management
First, confirm the diagnosis before treating: obtain the vulvar punch biopsy as above; do not start chronic ultrapotent corticosteroid therapy for a lesion that has not been examined and, where atypical or unresponsive, biopsied.
- Complications
Squamous cell carcinoma of the vulva: up to 5% of genital lichen sclerosus undergoes malignant transformation to squamous cell carcinoma, the disease's most serious long-term complication, making lifelong surveillance essential.
- Prognosis
Good symptomatic control with treatment: ultrapotent topical corticosteroid therapy controls symptoms and halts progression of sclerosis in most patients when started early and maintained.
- Prevention
Early biopsy-confirmed diagnosis and prompt corticosteroid initiation: starting clobetasol propionate 0.05% ointment as soon as the diagnosis is confirmed limits progression to labial fusion, clitoral phimosis, and introital stenosis.
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Related articles
- Vulvar cancer
Vulvar cancer is usually squamous cell carcinoma of the vulvar skin, arising either from high-risk HPV infection (younger, premenopausal patients) or from long-standing lichen sclerosus (older, postmenopausal patients), and is confirmed by punch biopsy of any persistent vulvar lesion or unexplained pruritus; treatment is surgical, with radical vulvectomy and inguinal lymphadenectomy for invasive disease.
- Lichen planus
Lichen planus is an idiopathic, T-cell-mediated inflammatory disease producing pruritic, violaceous, flat-topped polygonal papules on flexor skin and lacy Wickham striae on mucosa, and it is a clinical diagnosis built on the classic 6 P's. First-line treatment is discontinuing any causative drug plus high-potency topical corticosteroids, reserving systemic corticosteroids for severe widespread disease.
- Vulvovaginal candidiasis
Candida vulvovaginitis presents with vulvar itching, burning, and thick curdy white discharge at a normal vaginal pH; uncomplicated disease clears with a single 150 mg oral fluconazole dose or a short topical azole course, while complicated or recurrent disease needs a longer induction course followed by suppressive therapy.
- Dyspareunia
Dyspareunia is triaged by pain timing and location, not treated as one disease: superficial pain at the introitus on entry points to vulvovaginal atrophy, infection, vulvodynia, or vaginismus, while deep pain with penetration points to endometriosis, pelvic inflammatory disease, or a pelvic mass, and each branch has its own diagnostic and treatment pathway.