Vulval disorders: lichen sclerosus and vulvodynia

• The vulva has a small set of dermatoses that behave differently from the same condition elsewhere on skin: lichen sclerosus, lichen planus, eczema, contact dermatitis, psoriasis and vulvodynia. Each looks like the others when badly inflamed, so the diagnosis is clinical first and biopsy-led when the picture is atypical.
• Lichen sclerosus is the most important vulval dermatosis. It causes porcelain-white, fragile skin, loss of the labial architecture and painful fissures, and it carries a lifetime risk of vulval squamous cell carcinoma of roughly 2 to 5 percent.
• The standard treatment is ultra-potent topical glucocorticoid, usually clobetasol propionate 0.05% ointment once daily for 3 months, then a proactive maintenance regimen. About 60 to 70 percent of patients are symptom-free after that initial course.
• Vulvodynia is vulval pain lasting 3 months or more with no visible cause. It affects about 8 to 10 percent of women and is the commonest cause of painful intercourse in premenopausal women.
• Paget disease of the vulva is rare adenocarcinoma of apocrine skin, presenting as a sharply demarcated, eczema-like plaque in postmenopausal women, and it needs surgical excision.

By the end of this page you should be able to:

1. Recognise lichen sclerosus at its early inflammatory and late sclerotic stages and list its complications.
2. Explain the difference between lichen sclerosus, mucosal lichen planus, vulval eczema and lichen simplex chronicus.
3. Apply the ISSVD classification of vulvar pain and classify a case of vulvodynia by location, provocation, onset and temporal pattern.
4. Plan the initial and maintenance topical steroid treatment of lichen sclerosus and the follow-up needed for cancer surveillance.
5. List the causes of vulval pain with and without visible skin change, and describe when a biopsy is required.
6. Recognise extramammary Paget disease of the vulva and outline its investigation and treatment.

For practical purposes, the common disorders fall into four groups.

Inflammatory dermatoses with a vulval predilection. Lichen sclerosus, mucosal lichen planus and lichen simplex chronicus tend to choose the vulva and usually affect no other body site. These are the conditions usually meant by "vulval skin disorders". ^[3]

General dermatoses that involve the vulva. Atopic dermatitis, psoriasis, hidradenitis suppurativa and vitiligo involve the vulva as one of several sites. Unless the vulval component is treated as part of the whole disease, it is missed.

Vulvar pain without visible skin change. Vulvodynia is burning or rawness of the vulva lasting 3 months or more with no identifiable cause. It is classified by the 2015 ISSVD, ISSWSH and IPPS consensus into localized or generalized, provoked or spontaneous or mixed, primary or secondary onset, and intermittent or persistent or constant or immediate or delayed temporal pattern. Vulvar pain caused by a specific disorder (infection, inflammation, neoplasm, nerve damage, trauma, surgery or hormonal deficiency) is not vulvodynia and must be excluded first. ^[3]

Neoplastic disease of vulval skin. Vulvar squamous carcinoma (often arising in lichen sclerosus or differentiated vulvar intraepithelial neoplasia), vulvar melanoma, and extramammary Paget disease. These are covered in the vulvar and vaginal cancer page; Paget disease is summarised below because it mimics eczema. ^[4]

Lichen sclerosus is the commonest clinically significant vulval dermatosis in adult women. Its true prevalence is unknown because mild disease is rarely biopsied and many women do not present. In one gynaecology practice cohort the prevalence reached 1.7 percent of women, and reports range far higher in postmenopausal groups in some series. The same review estimates the incidence of childhood lichen sclerosus at 0.04 to 0.06 percent. Women are affected far more than men, with extragenital disease making up only about 10 percent of cases. Presentation is bimodal, peaking before puberty and in the perimenopausal or postmenopausal years, which is why hormonal influences are suspected. ^{[1] [4]}

There is a long diagnostic delay. About 4.6 years pass on average between the first symptom (itch, pain, dysaesthesia or defaecation discomfort) and the diagnosis, so it is worth asking and examining specifically. ^[1]

Mucosal lichen planus affects men and women at any age. Lichen planus of all types occurs in 0.5 to 1 percent of the population. Vulvovaginal mucosal lichen planus chooses Caucasian women aged 40 to 60, often alongside oral disease; about half of women with oral mucosal lichen planus have genital involvement, which is why the genital area should be examined in every patient with oral lichen planus. ^{[1] [3]}

Vulval eczema and lichen simplex chronicus are probably the commonest vulval complaints overall, but neither has a reliable population estimate. In a German dermatology practice cohort 45 percent of women with atopic dermatitis reported at least one episode of genital involvement. ^[1]

Vulvodynia affects an estimated 8 to 10 percent of women of all ages worldwide, with about 16 percent of US women reporting it at some point in life and 7 to 8 percent by age 40. A Spanish study found 13 percent lifetime prevalence. It is the commonest cause of pain during sexual intercourse in premenopausal women, yet diagnosis is delayed: nearly 40 percent of affected women in a US population study did not seek treatment, 60 percent of those who did saw three or more doctors, and in Europe only 10 to 25 percent of patients receive the correct diagnosis on the first gynaecology visit. ^[3]

Extramammary Paget disease of the vulva accounts for about 1 to 2 percent of vulval neoplasms and mainly occurs in postmenopausal women over 60, with a mean age at diagnosis around 70 in the reviewed literature. ^[4]

Lichen sclerosus is multifactorial. A genetic predisposition is clear from the strong association with other autoimmune diseases, and the condition is not primarily hormonal despite its age distribution. Sex hormone levels in affected women are usually normal, and the response to topical oestrogen or testosterone is poor; current evidence does not support restoring hormones as a treatment. Reported triggers include local trauma (the Koebner response: surgery, piercings, chronic urine exposure), infections (viruses and Borrelia in some series), and chronic irritants. In women, urine incontinence is a particularly common precipitant. ^[1]

Lichen planus is an autoimmune cytotoxic T-cell attack on basal keratinocytes, triggered by viral infection, drugs, vaccination or trauma in predisposed patients, and is associated with other autoimmune disease and hepatitis C in some populations. ^[1]

Vulval eczema arises from contact with irritants (soaps, perfumed products, antiseptic wipes, laundry detergent, sanitary pads, urine, sweat), contact allergens (fragrances, preservatives, rubber accelerators, nickel, topical antibiotics and anaesthetics), or atopy. Lichen simplex chronicus is usually a chronic, localised manifestation of atopic dermatitis maintained by the itch-scratch cycle. ^[1]

Vulvodynia is multifactorial. Potential associated factors listed by the 2015 consensus include genetics, hormonal factors (including pharmacological hormonal change), inflammation, musculoskeletal and pelvic floor dysfunction, neurologic mechanisms (central sensitisation or local neuroproliferation), psychosocial factors, structural defects, and comorbid pain syndromes such as fibromyalgia, irritable bowel syndrome, painful bladder syndrome and temporomandibular disorder. ^[3]

Paget disease of the vulva probably originates from apocrine gland cells or pluripotent keratinocytes and may be primary (cutaneous) or secondary to an anorectal, urothelial or other adenocarcinoma spreading to the epidermis. An underlying internal malignancy is sought in every case. ^[4]

In lichen sclerosus the early lesion is a lichenoid interface dermatitis: a dense infiltrate of mostly CD4-positive and CD8-positive T-cells in the upper dermis attacking basal keratinocytes, in a Th1 cytokine milieu. Later, pro-sclerotic factors predominate, leading to subepidermal glassy oedema, epidermal atrophy, loss of elastic fibres and dilatation and loss of dermal vessels (haemorrhage is common). Inflammation plus vascular sclerosis generates oxidative stress and down-regulation of tumour suppressor genes, which is believed to contribute to carcinogenesis. ^[1]

Mucosal lichen planus is a cytotoxic T-cell attack on basal keratinocytes producing saw-tooth acanthosis, hypergranulosis and Civatte bodies early, with variable atrophy, hyperkeratosis, erosion and scarring later. Vaginal involvement means discharge, and chronic scarring can produce stenosis. ^[1]

For vulvodynia, current neurobiological thinking treats it as a primary chronic pain condition with altered central pain processing, sometimes with local neuroproliferation, rather than ongoing tissue damage. That is why a physical sign alone neither proves nor excludes it, and why pain management and psychosocial care are central. ^[3]

The biopsy of lichen sclerosus changes with stage. Early disease shows a lichenoid inflammatory infiltrate with single-cell basal necrosis; late disease shows an atrophic epidermis, a thick glassy band of dermal hyalinisation, a sparse infiltrate and absence of elastic fibres in that zone. The diagnosis is usually clinical, but biopsy is recommended for equivocal findings, treatment failure or to exclude malignancy. ^[1]

Under the microscope Paget disease shows large Paget cells with pale cytoplasm scattered in the epidermis, with a pagetoid distribution. These cells are glandular and stain for mucin and for markers such as CK7 and GCDFP-15; staining helps separate primary from secondary disease. ^[4]

Symptoms. Itching is the dominant symptom, sometimes severe enough to disturb sleep, with burning, soreness, dysuria and dyspareunia. Chronic pain and defaecation discomfort are common. Some patients are asymptomatic and the disease is found incidentally.

Signs. Early disease shows shiny erythema, often in a figure-of-eight pattern around the vulva, perineum and anus. Persistent inflammation produces porcelain-white, thickened or crinkled skin, purpura and haemorrhagic bullae, and painful fissures. Late disease produces irreversible architectural change: loss of the clitoral hood, resorption and fusion of the labia minora, narrowing of the vaginal introitus and, in men, phimosis. The vagina itself is not affected. ^[1]

Children. Prepubertal girls present with itching, soreness, vulvar soreness and sometimes constipation or bleeding. The distinction from abuse or infection is clinical and often needs a specialist.

Painful or pruritic erosions with Wickham striae are characteristic, with variable discharge, atrophy or hyperkeratosis and chronic ulceration. It is a disorder of mucosal rather than hair-bearing skin, so the vaginal epithelium can be involved. Scarring, stenosis and adhesions are late complications. Severe disease is painful rather than merely itchy. ^[1]

Erythematous, sharply or diffusely demarcated lesions of the labia minora and majora, with oedema in the acute phase and lichenification in the chronic phase, with severe pruritus. Irritant and allergic contact dermatitis localise to the sites of exposure (mon pubis, groin, perineum); atopic dermatitis often involves groin and flexures. ^[1]

Circumscribed, hypertrophic, lichenified and sometimes eroded papules coalescing into nodules and plaques, usually on the labia majora, mons and perineum. Scaling and exudation are variable. The itch-scratch cycle must be broken for treatment to work. ^[1]

Pain, usually burning, with no visible skin change: discomfort on contact or entry, or spontaneous pain unrelated to intercourse. There is often co-occurring pelvic floor muscle spasm, tenderness on cotton-swab mapping of the vestibule, and psychosexual difficulty. Vulvodynia is not a skin disease as such; ruling out skin, infection and neoplasm is the first step. ^[3]

A sharply demarcated, erythematous, scaly or weeping plaque that looks like chronic eczema, most often on the labia majora of a woman over 60, resistant to steroid treatment, with itching, burning or pain. Multifocality is common, showing the classic "strawberry and cream" appearance in some lesions. ^[4]

A common trap is to treat repeated "recurrent candidiasis" in a postmenopausal woman with steroids and antifungals without examining the skin. Candidal superinfection genuinely occurs in 9.5 to 11.5 percent of biopsied lichen sclerosus and lichen planus, more often when a flare breaks through a previously stable regimen; in one dermasilk trial candidiasis occurred in 9.5 percent and in a retrospective individualised-steroid series in 6.2 percent. If a flare resists usual therapy, take a swab, and if necessary a biopsy, before escalating. ^[5]

Most vulval skin disorders are diagnosed clinically. Biopsy is reserved for atypical appearance, treatment failure, lesions that do not fit, or any suspicion of neoplasia. A 3 to 4 mm punch under local anaesthetic is usually adequate; the histopathology report must state the anatomic site, because keratinising squamous epithelium on the labium majus is normal while the same finding in the vestibule is metaplasia. ^[1]

Swabs and cultures are useful where infection is suspected: bacterial culture where erythema with pustules or fissures is present, fungal culture for suspected candidal superinfection (culture is best taken from skin scrapings rather than a vulvovaginal swab, and results take over 2 weeks), and herpes simplex polymerase chain reaction when ulcers are present. Wet mount and molecular tests are rarely useful for skin disease. ^[5]

Dermoscopy and vulvoscopy help with unclear growths. Acetic acid testing, patch testing and Wood's lamp examination are used when contact dermatitis, erythrasma or tinea is suspected. ^[1]

Paget disease is diagnosed on a vulvar excisional biopsy that must be generous enough to include the edge of normal skin. Clock mapping (intensive preoperative biopsy sampling) helps define microscopic extent. Work-up includes pelvic ultrasound, magnetic resonance or computed tomography, breast examination and, in selected cases, colposcopy and cystoscopy to separate primary from secondary disease. ^[4]

For vulvodynia the current 2015 ISSVD, ISSWSH and IPPS taxonomy is the standard. Vulvar pain lasting 3 months or more without an identifiable cause is vulvodynia; vulvar pain caused by a specific disorder is listed under specific causes (infection, inflammation, neoplasm, neurologic, trauma, iatrogenic, hormonal). Vulvodynia is then described as localized (for example vestibulodynia, clitorodynia), generalized or mixed; provoked (insertional or contact), spontaneous or mixed; primary or secondary onset; and intermittent, persistent, constant, immediate or delayed. ^[3]

For vulval pain in general a useful bedside algorithm is:

Wash with water alone, or with a pH-neutral, hypoallergenic cleanser if soap is preferred. Avoid perfumed products, wet wipes, tight synthetic clothing and repeated shaving. Wear light, loose underwear. Sitz baths with astringent tanning agents can help during flares. In the acute stage use astringent creams; in the chronic stage use ointments, which are less allergenic, applied regularly in small amounts. Late stenosis can be managed carefully with dilators; support groups help. ^[1]

The standard of care is an ultra-potent or potent topical glucocorticoid, an ointment rather than a cream, combined with emollients. The gold standard in practice is clobetasol propionate 0.05% ointment applied as a thin layer (about a fingertip unit per affected area) once daily for 3 months. Less potent class III steroids such as mometasone furoate 0.1% can be used, and are preferred in children and in pregnancy, where absorption is to be avoided. About 60 to 70 percent of patients achieve complete remission of symptoms after that course, usually within less than 10 days for the first visible improvement. A head-to-head trial found mometasone 0.1% once daily similarly effective after 12 weeks, with an improvement of at least 75 percent in subjective scores in 59 percent (clobetasol) versus 67 percent (mometasone) and in objective scores in 37 percent versus 48 percent. ^{[2] [1]}

Emollients (paraffin-based ointments) are basic therapy alongside the steroid, applied regularly to protect the barrier. ^[2]

After the 3-month course, taper to an individualised maintenance frequency, for example twice weekly, because complete remission without some maintenance is rare. Many clinicians use a "proactive" regimen: treat every flare promptly and keep a minimum weekday dose. ^[1][2]

Doses cited in different regimens vary. The German S3 guideline and the British Association of Dermatologists 2018 guideline agree on clobetasol 0.05% as first line, while differing on exact tapering; the EuroGuiDerm 2023 guideline advises the same 3-month initial course; the ISSVD consensus, applied through the 2015/2019 vulvar pain taxonomy, structures assessment of associated disease; and the Royal College of Obstetricians and Gynaecologists Green-top guideline on vulval skin disorders sets out similar first-line and follow-up principles. Because regimens are agreed consensus rather than single trials, follow your national guideline and local practice. ^{[2] [3]}

Topical calcineurin inhibitors (tacrolimus or pimecrolimus) are second-line, off-label, applied twice daily for 3 to 6 months, then tapered, when topical steroids are contraindicated or insufficient. UVA1 phototherapy, platelet-rich plasma, laser therapy (fractional CO2, Nd:YAG, Er:YAG) and photodynamic therapy appear promising but lack high-quality evidence; cryotherapy is generally not recommended. Systemic treatment with methotrexate or acitretin is reserved for severe disease. A vulvectomy does not cure, because lichen sclerosus often spreads to transposed skin. ^[1][2]

General measures, as above. Flares are treated with clobetasol propionate ointment twice daily long term, then proactive maintenance. Intravaginal disease can be treated with ointment or foam (for example budesonide rectal foam) applied with a fingertip or applicator. Superpotent steroids are often combined with topical calcineurin inhibitors, aloe vera or tocopherol off-label. Hypertrophic plaques may need intralesional glucocorticoid (painful). Dilators and estriol help prevent stenosis. Systemic treatment (systemic glucocorticoid induction, acitretin or isotretinoin, methotrexate, ciclosporin) is used in severe or refractory cases. Photodynamic therapy has some evidence in small studies for vulvovaginal disease. ^[1]

Guidelines differ here: there is no German guideline for lichen planus alone, the EuroGuiDerm and European Academy of Dermatology and Venereology S1 guideline (2020) gives general orientation, and the British dermatology literature gives practical advice close to the lichen sclerosus pathway. ^[1]

Remove the trigger. Prescribe a class II topical glucocorticoid such as prednicarbate ointment for 2 to 4 weeks with tapering frequency, which is often sufficient. In atopic diathesis topical calcineurin inhibitors can be used on-label. Severe, widespread eczema may require systemic therapy for atopic dermatitis. Supportive care and bland barrier therapy are more important here than anywhere else. ^[1]

There is no single treatment with strong evidence, and individualisation is key. A three-step approach is widely used: step one combines vulval care measures, dietary advice, topical (low-dose local anaesthetic, sometimes short-course ultra-potent steroid for hidden inflammation) and oral medication, pelvic floor physiotherapy and cognitive behavioural and psychosexual therapy. Step two adds minimally invasive neuromodulation such as injections, radiofrequency or neurostimulation. Step three reserves surgery (vestibulectomy, pudendal neurolysis) for refractory localised provoked disease in selected patients. Pain management and psychosocial care should start early. Placebo has been shown to be as effective as many medications used for vulvar pain, so expectation management matters. ^[3]

Surgery is the mainstay: wide local excision in most patients, or hemivulvectomy, partial or total simple vulvectomy when needed, with skinning vulvectomy for selected flat lesions. Re-excision is needed when margins are positive. Preoperative clock mapping helps define microscopic extent, and inguinofemoral node assessment (lymphadenectomy or sentinel node biopsy) is considered when lesions are invasive. Older or frail patients and those with unresectable disease may be treated with radiotherapy (up to about 61 Gy in total for gross disease, 63 Gy for radical regimens in the reported series), or with topical imiquimod 5%, 5-fluorouracil, photodynamic therapy or laser. Chemotherapy (usually cisplatin or carboplatin-based, paclitaxel or docetaxel) is reserved for metastatic disease. ^[4]

Lichen sclerosus can cause irreversible architectural change (labial fusion, clitoral burial, introital stenosis), recurrent fissures, dyspareunia, voiding difficulty and secondary constipation in children. Malignant transformation to vulval squamous carcinoma is the complication that drives follow-up. Women affected by lichen sclerosus carry a lifetime risk of developing vulval squamous cell carcinoma of roughly 2 to 5 percent, and up to 65 percent of vulval carcinomas occur in a background of lichen sclerosus. Candidal superinfection is also common on previously stable disease. ^[1][5]

Lichen planus can scar, produce vaginal stenosis and adhesions, and is thought to carry a modestly increased risk of vulval squamous carcinoma in severe erosive or hyperkeratotic disease. ^[1]

Vulvodynia itself is not premalignant but is disabling, with serious consequences for sexuality, intimacy, self-esteem and quality of life. ^[3]

Paget disease is malignant but slow; about 10 percent progress to invasive disease and nodal or distant spread. Local recurrence is common (23 to 73 percent in the reported series) because of multifocal microscopic disease and frequent positive margins, despite a 5-year survival of roughly 75 to 90 percent. ^[4]

With prompt and adequate ultra-potent steroid treatment, most patients with lichen sclerosus have good symptom control and structural progression is slowed, although architectural change already present is often permanent. Architectural change already present is usually irreversible.

Vulvodynia has a variable course: spontaneous remission, persistent disease and relapse are all common. Early pain management and psychosexual support improve outcome. ^[3]

Paget disease has an excellent prognosis if treated before invasion, but needs long follow-up because recurrence is frequent. ^[4]

There is no way to prevent lichen sclerosus. Reducing avoidable vulvar irritation (harsh washing, tight clothing, chronic urine exposure) and maintaining steroid remission probably reduce progression and scarring. Patient self-examination, encouraged by the treating team, is the practical form of screening. ^[2]

For vulval neoplasia, the wider strategies (HPV vaccination, smoking cessation, chronic disease control) apply; all suspicious persistent lesions should be biopsied. ^[4]

Children. Childhood lichen sclerosus has an incidence of 0.04 to 0.06 percent. Potent or ultra-potent glucocorticoids are first-line, recognising that not all preparations are licensed for all ages, with specialist follow-up through puberty, because disease can remit but may recur. Surgery in children is generally avoided except for individual functional problems. Mucosal lichen planus is rare in children. ^[2]

Pregnancy. Class III steroids are generally preferred in pregnancy because absorption (and any risk of fetal growth restriction) is avoided; topical hormones are not used for lichen sclerosus. Vulval eczema tends to improve but pregnancy is a risk period for candidal superinfection. ^[1][2]

Older adults. Lichen sclerosus and Paget disease both cluster in older women. Atrophy and comorbidity complicate topical treatment, ointment vehicles are better tolerated, and cognitive or dexterity limitations may make adherence harder. ^[4]

Immunocompromise. Candidal superinfection and other infection are more frequent; topical steroid treatment may be less effective. ^[5]

• Potent topical steroids and emollients are cheap and widely available, so lichen sclerosus and eczema can be treated effectively even without specialist services.
• Biopsy, not empiric treatment, is what separates a dermatosis from Paget disease; if biopsy is not available, any eczema-like vulval plaque that has not responded after 4 to 6 weeks of steroid should be referred.
• A 3 to 4 mm punch biopsy, taken under local anaesthetic, is a low-cost, high-value test that should be taught in every setting.
• Many patients can be followed by photographs taken on a phone, with 6-monthly review, where clinic visits are costly.
• In vulvodynia, pelvic floor physiotherapy, vulval care measures and cognitive behavioural support cost less than medication or surgery and are a reasonable first-line plan.

• Lichen sclerosus is the most important vulval dermatosis: white, fragile skin, painful fissures, architectural change, and a 2 to 5 percent lifetime risk of vulval squamous carcinoma.
• In women lichen sclerosus spares the vagina. If vaginal involvement is present, think lichen planus.
• Ultra-potent topical glucocorticoid (clobetasol 0.05% ointment) once daily for 3 months, plus emollients, is the standard of care; then proactive maintenance.
• About 60 to 70 percent of patients are symptom-free after the initial 3-month course.
• The treatment of lichen sclerosus lowers the risk of malignant transformation.
• Follow up: every 3 to 6 months at first, then 12-monthly once stable, with careful inspection and biopsy of any new or non-healing lesion. ^[2]
• Topical hormones are not used for lichen sclerosus, despite the age distribution of the disease.
• Vulvodynia is defined as pain lasting 3 months or more with no identifiable cause; drugs alone are rarely enough, and pelvic floor physiotherapy and psychosexual care come first.
• Paget disease of the vulva looks like eczema that will not clear; biopsy any treatment-resistant plaque, especially in women over 60.
• Candidiasis in a woman with lichen sclerosus is usually superinfection, not failed treatment; swab or biopsy before escalating.

Vulvar and vaginal cancer; vulvovaginal candidiasis; chronic pelvic pain; menopause and hormone therapy; psoriasis; atopic dermatitis; sexual dysfunction; dermatology physical examination. (No links as these topics exist only by title in the MedGrasp library.)