Lichen Sclerosus -Information for Referring Doctors

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Information for Referring Doctors

One of our aims at the Dermatology/Vulval conditions clinic is to share the care of patients as much as possible with the referring doctor.  Improving the knowledge and understanding of vulval lichen sclerosus is also an important goal. As such we have provided this information sheet to try to assist doctors caring for these women.

What is Lichen Sclerosus? (LS)

Lichen sclerosus is an uncommon condition in which a characteristic type of inflammation of the dermis causes a distinctive skin appearance. The cause is not known, but is likely to be autoimmune.
 
LS can occur anywhere on the body but most commonly affects anogenital skin, especially in females.
In men and boys the penis (particularly if uncircumcised) and perianal area may be affected, and phimosis and urethral stenosis may develop.
 
In women and girls the vulva and perianal area are involved but the vagina is spared. Vulval lichen sclerosus has previously been known by a number of names, including leukoplakia and vulval dystrophy.  It can occur at any age but is most commonly seen around the time of menopause or before puberty.

If the vagina is involved, lichen planus is usually the diagnosis. The vulval appearance can be similar but usually there is much more mucosal reddening. An erosive form exists and may involve the mouth also. Lichen planus can coexist with lichen sclerosus.
 
Symptoms and Signs, Risks and Associations of Vulval Lichen Sclerosus

General health remains normal.  Itch and soreness are the main symptoms but sometimes the patient is asymptomatic. Initially the skin is red and inflamed but later becomes white, shiny, crinkly and atrophic. Sometimes patches of affected skin can be quite lichenified. Erosions, bullae or purpura may occur.
 
In longstanding disease there is usually an alteration of the normal anatomy of the vulva. The labia minora may shrink or be completely resorbed onto the labia majora, and the opening to the vagina may become narrowed, resulting in difficult and painful intercourse.  The clitoris can become buried due to scarring of the clitoral hood. The vagina is unaffected.
 
Longitudinal studies suggest a roughly 4% (reports range from 1-11%) risk of development of squamous cell carcinoma within areas affected by LS.  It is likely the risk is highest in those with a hyperkeratotic tendency, and review more frequently is suggested for these patients.
 
There may be a family history of this skin disease and an increased risk of other autoimmune diseases such as vitiligo, alopecia areata, diabetes mellitus , pernicious anaemia, coeliac disease or collagen disorders.  In adult women it can sometimes be associated with autoimmune thyroid dysfunction. Fasting BSL/HbA1c, FBE, TFTs, thyroid antibodies, parietal cell antibodies, and coeliac disease tests are recommended according to the clinical context. . We have asked your patient to discuss monitoring the need for these tests with you. 
 
Aims of Treatment for Vulval Lichen Sclerosus
Control of symptoms such as itch, soreness, dyspareunia 
Minimization of scarring and alteration of normal vulval architecture 
Surveillance for squamous cell carcinoma 
Reduction in risk for SCC (data is uncertain regarding the effect of corticosteroid and reduction in cancer risk, but the reduction in chronic trauma through scratching is likely in itself to reduce cancer risk). 

Management

Genital hygiene 
Avoidance of irritants and soaps is most important.  Daily pads are discouraged.  Sexual lubricants are often necessary. Moisturizers and/or barrier ointments (such as sorbolene ,dermeze or zinc paste) are encouraged on a daily basis if the skin is particularly dry and especially if there is incontinence. A detailed information sheet is supplied to patients.

Topical steroids 
The most successful treatment is steroid ointment (typically Diprosone OV or advantan ointments).  These are used once or twice daily for the first month, usually daily in the 2nd month, then at reducing levels depending on symptoms and the appearance of the skin. A 30g tube should last at least 3 months and is quite safe at this level of usage. Maintenance treatment is often only x1-2 applications per week. There may be a burning sensation when steroid ointment is first applied, but this usually disappears within 10 minutes. If it persists, the steroid should be stopped, as an irritant or allergic contact dermatitis may be the cause. Review at the vulval clinic is recommended.
 
In general, it is preferable to use medication often enough to avoid all symptoms.  Treatment based on the presence of symptoms can risk ongoing scarring. For some women, progressive LS can occur without symptoms. Also, despite resolution of itch during treatment, there can sometimes still be mild active disease. Pulsed treatment, such as weekly, has very little risk of steroid induced skin atrophy and is often recommended as the maintenance dose.
 
Topical androgens have previously been used for LS, but studies have shown that their benefits are not better than placebo.

Symptom flares 
When symptoms have been controlled and a maintenance level of medication has been successful for some time, acute symptoms of LS may occasionally occur.  Treatment frequency will need to be increased (usually daily) for a week or more.  It is advisable to exclude candidal, bacterial and, if the skin is split, herpes infections. New irritants should be enquired about especially new use of pads and panty liners.

Most importantly we ask the woman to show us where she applies the medication. In the clinic we encourage the USE OF A MIRROR to check together the areas that need treatment. Many women have never looked at their vulva. Often the ointment is applied in a general area but not to the problematic area.

Followup 

Surveillance of lichen sclerosus and lichen planus needs to be lifelong.
Once the condition is controlled, we recommend 3-6 monthly review in the initial 1 to 2 years, to observe the clinical pattern of LS. Well controlled LS shows persistence of the anatomical changes to the labia and clitoral areas and pale skin areas (sometimes reddened smooth areas also), but the skin will be smooth. At this stage review can be reduced to x1-2 annually lifelong, and women will be encouraged to self monitor using a mirror more frequently. 
 
Where LS has a tendency to be lichenified, followup will need to remain at 3-6 monthly. At review, visual or palpable changes should be specifically sought. These include thickening, white roughened patches, induration, lumps or non-healing ulcers or fissures. Any suspicious areas require biopsy to exclude SCC.
 
Occasionally architectural changes such as introital narrowing require surgical treatment, but ongoing topical treatment will continue.

The Role of the Vulval/Dermatology Clinic.

The aim of the clinic is to make or confirm the initial diagnosis of LS, and then to gain symptomatic control of the disease.  Once this is achieved we hope it will be possible for the referring doctor to perform ongoing surveillance through vulval examination at least 6 monthly. If there are any problems with this, or if there are suspicious areas, please be in touch. Urgent review or advice can be arranged with the clinic. MSHC operates also on a walk in basis for acute symptoms. Specialist advice can be readily sought in this way.

DISCLAIMER:
This fact sheet is designed to provide you with information on Lichen Sclerosus -Information for Referring Doctors. It is not intended to replace the need for a consultation with your doctor. All clients are strongly advised to check with their doctor about any specific questions or concerns they may have. Every effort has been taken to ensure that the information in this pamphlet is correct at the time of printing.

Last Updated 2012