QuestionSBA
QuestionChoice
This woman has features of lichen sclerosus, an inflammatory skin condition that often affects the vulva. It appears as hypopigmentation of the vulval and perianal skin, characteristically appearing in a "figure of eight" distribution. In severe cases, thickening and scarring occurs, which can completely obliterate vulval structures such as the labia minora and clitoris. Fissures are common, particularly around the posterior fourchette (the thin, v-shaped tissue joining the labia minora inferiorly). Fissures can easily tear and cause discomfort and bleeding.
 

 Vulval biopsy should be performed to confirm the diagnosis of lichen sclerosus. Lichen sclerosus carries an increased risk of vulval intraepithelial neoplasia (VIN) and squamous cell carcinoma. In this case in particular, the distribution of the hypopigmentation is not well-demarcated or symmetrical, raising a concern of pre-cancerous or cancerous changes.
49792
a
Vulval biopsy
QuestionChoice
A vulvovaginal swab for culture and sensitivity would be indicated if there was any symptoms or signs of infection such as a new vaginal discharge or pelvic pain.
49796
e
Low vulvovaginal swab for culture and sensitivity
QuestionChoice
Topical oestrogen therapy is used to treat vulvovaginal atrophy. Whilst vulvovaginal atrophy presents in a similar way to this woman, the "figure of eight" pattern, the "porcelain white" appearance and the presence of scarring is more indicative of lichen sclerosus than vulvovaginal atrophy. Atrophy typically presents with thinning of the vulval tissues and does not often cause fusion of vulval structures.
49794
c
Topical oestrogen therapy
QuestionChoice
Topical ultrapotent steroid cream is the first-line management for lichen sclerosus; however, a biopsy should be performed first to confirm. The patient should have been off steroid therapy for at least three weeks prior to performing a biopsy.
49795
d
Topical vaginal moisturiser
QuestionChoice
Topical ultrapotent steroid cream is the first-line management for lichen sclerosus; however, a biopsy should be performed first to confirm. The patient should have been off steroid therapy for at least three weeks prior to performing a biopsy.
49793
b
Topical ultrapotent steroid cream
Concept
Chapter
# Summary
  
 
Lichen sclerosus is an inflammatory skin condition that predominantly affects the genital and anal areas, characterized by white patches which may scar. It frequently presents itself with symptoms of itchiness and pain, and is more common in females than males. Key investigations for this condition include clinical evaluation, skin biopsy, and possibly blood tests. Management strategies often include topical steroids, avoidance of soaps in affected areas, and use of emollients to alleviate dryness and itching.
  
 
# Definition
  
 
Lichen sclerosus is an inflammatory dermatological condition. It predominantly affects the genital and anal regions of the body, but it can present elsewhere. There is a noted predilection for this condition in females compared to males. The subtype, vulvar lichen sclerosus, specifically involves the inner vulva.
  
 
# Aetiology
  
 
The cause of lichen sclerosus is currently unknown, but it is likely multifactorial. Potential contributing factors could include autoimmune reactions, genetic predisposition, and hormonal factors. Some studies suggest a possible link with previous skin damage or trauma.
  
 
# Signs and Symptoms
  
 
- White patches on the skin which may progress to scarring. 
- Itchiness and pain over patches, which may be exacerbated during urination or sexual intercourse.
- Bleeding over patches, especially following sexual intercourse or passing of bowel motions. 
  
 
  
 
# Differential Diagnosis
  
 
The differential diagnosis for lichen sclerosus includes other dermatological conditions such as:
  
 
  - Lichen planus: Characterised by purple-looking, itchy, flat-topped bumps, and white lacy patches in the mouth or on the skin.
  
  - Psoriasis: Manifests as red patches with silver scales, typically on the scalp, elbows, knees, and lower back.
  
  - Vitiligo: Presents as patchy loss of skin color, usually first on sun-exposed areas of the skin.
  
 
# Investigations
  
**Bedside:**

- Vulval examination: This should be enough to diagnose lichen sclerosus, if characteristic lesions are present. 


No **blood tests** are required for diagnosis, but may on occasion be used to rule out other autoimmune conditions if there is clinical uncertainty. However, keep in mind that serology for autoimmune conditions often has low specificity. 

No **imaging** is required.

**Invasive:**

- Skin biopsy: May be used to confirm the diagnosis. 
  
 
# Management
  
 
The management of lichen sclerosus includes:
  
 **Conservative:**
 
 - Avoidance of soaps in the affected areas to prevent further irritation.
 - Avoid tight clothing, rubbing or scratching. 
 - Topical emollients to relieve dryness and soothe itching. 
 
**Medical:**


 - Topical corticosteroids (most often potent steroids, such as dermovate) to reduce inflammation and itching.
 - Topical cacineurin inhibitors (e.g. Tacrolimus) may be used in addition to steroids. However, they can cause burning and discomfort in the first few days of application. 
 - Topical oestrogen cream or pessaries for postmenopausal women affected by atrophic vulvovaginitis, caused or exacerbated by scarring of lesions.  
 - Oral treatment with corticosteroids, retinoids, methotrexate or ciclosporin may be indicated in severe cases that do not respond to topical therapies. 

**Surgical:**

Whilst surgical management is not usually performed, it can be used in cases of severe adhesions and advanced scarring that impacts continence and sexual intercourse, where conservative and medical measures have not helped.
Surgery would also be indicated in cases of squamous cell carcinoma (see complications). 


# Complications

- Infections: Namely Candida albicans, Staphylocuccus aureus, Herpes simplex, predisposed by breaks in the skin. 
- Squamous cell carcinoma: Affects up to 5% of patients with vulval lichen sclerosus. 
- Worsening constipation: Often as a result of pain or discomfort while passing bowel motions. 
 
 
# NICE Guildelines

[Click here for NICE Guildelines on assessment of vulval itching](https://cks.nice.org.uk/topics/pruritus-vulvae/diagnosis/assessment/)


# References
  
 
[BASHH 2014 UK National Guideline on the Management of Vulval Conditions](https://www.bashh.org/documents/UK%20national%20guideline%20for%20the%20management%20of%20vulval%20conditions%202014.pdf)

[DermNet](https://dermnetnz.org/topics/lichen-sclerosus)

[NHS UK](https://www.nhs.uk/conditions/lichen-sclerosus/)
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Lichen sclerosus
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Gynaecology
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Lichen sclerosus
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Vulval cancer
Vulval biopsy
9995
A 66-year-old woman presents with sharp pain on defecation and occasional blood on toilet paper. She denies weight loss or changes in bowel habits.


She has a past medical history of type 2 diabetes which is well-controlled on metformin, hypertension and eczema.

On examination, her abdomen is soft and non-tender. Genital exam reveals smooth, dry vulva with porcelain-white shiny areas, particularly on the left. The labia minora and clitoris are not visible, and the labia majora are thickened with red patches. Linear lacerations are present at the posterior fourchette and external anal sphincter.


Digital vaginal examination is not possible. Digital rectal examination reveals no masses and soft stool in the rectum.


What is the most appropriate next step?
a