British Association of Dermatologists' Guidelines for the Management of Lichen Sclerosus 2010

S.M. Neill; F.M. Lewis; F.M. Tatnall; N.H. Cox

Disclosures

The British Journal of Dermatology. 2010;163(4):672-682. 

In This Article

Complications

Malignancy

Squamous cell carcinoma (SCC) has been described predominantly in association with female genital LS and less commonly in penile LS. It is not associated with extragenital LS. Less commonly the malignancy is a verrucous carcinoma. Melanoma, basal cell carcinoma and Merkel cell carcinoma have all been reported rarely in patients with vulval LS but no studies suggest that there is an increased frequency of these tumours. There appear to be two pathogenetic mechanisms for vulval SCC: firstly, SCC in younger women is associated with the oncogenic human papillomavirus (HPV); and secondly, in older women, the association is with a chronic scarring dermatosis such as LS or LP with little, if any, evidence of a link with HPV.

Squamous Cell Carcinoma in Female Patients with Genital Lichen Sclerosus SCC arising within LS only occurs in anogenital disease. The risk is small, being < 5%.[17,23,38] However, histopathological examination of vulval SCCs indicates that about 60% occur on a background of LS.[39–41] LS may act as both an initiator and promoter of carcinogenesis by mechanisms that seem to be independent of HPV. Although there is little evidence for an important role for HPV in LS-associated SCC, there has been a suggestion that topical corticosteroid use may induce oncogenic HPV types. HPV may be found in vulval intraepithelial neoplasia (VIN) associated with LS.[42] SCC of the vulva should be managed by oncological gynaecologists experienced in this field as surgery has to be individualized according to the tumour size and location, particularly in early invasive disease.

Squamous Cell Carcinoma in Men with Genital Lichen Sclerosus An association between LS and penile SCC has also been reported.[43–45] Although histological evidence of LS can be found in about 40% of penile carcinoma specimens, the actual risk of this complication in any individual patient with LS is uncertain. Published data suggest that the risk is about 5%, similar to the figure suggested for female patients.[43] In a 10-year multicentre cohort of 130 male patients with genital LS, histological changes of SCC were found in eight, verrucous carcinoma in two and erythroplasia of Queyrat (in situ SCC) in one.[46]

The role of HPV in penile LS-associated SCC has also been debated. Some studies using polymerase chain reaction have documented a negligible frequency of HPV in LS,[47,48] but other studies have suggested a frequency of up to 33%.[49,50] An additional feature that has been linked with penile LS-associated SCC is the occurrence of a prominent lichenoid infiltrate on long-standing, chronic LS, suggesting disease reactivation.[51]

Scarring

Introital Narrowing This is rare, but, if significant and causing dyspareunia or difficulty with micturition, surgery may need to be considered. Part of the posterior vaginal wall is used in the reconstruction to prevent further adhesions and stenosis due to Koebnerization.[52]

Pseudocyst of the Clitoris Occasionally, clitoral hood adhesions seal over the clitoris and keratinous debris builds up underneath forming a painful pseudocyst. This requires a subtotal or total circumcision.[53]

Preputial Adhesions and Phimosis If subcoronal or transcoronal adhesions between the inner aspect of the prepuce and the glans persist despite adequate medical treatment, these will need to be treated surgically and a circumcision performed at the same time. Persistent phimosis will also require a circumcision. If the disease is still active at the time of surgery a topical steroid might be required to prevent Koebnerization and further scarring, particularly around the coronal sulcus.

Meatal Stenosis If this results in an impaired urinary stream, referral for urological assessment is advisable.

Sensory Abnormalities: Dysaesthesia

Vestibulodynia and Vulvodynia These conditions may occur after an inflammatory condition of the vulva or vestibule. Typically, the patient remains symptomatic despite objective clinical improvement or resolution of the skin lesions. Neuropathic pain does not respond to topical corticosteroids, and treatment must be directed to the eradication of the neuronal sensitization. Initially, 5% lidocaine ointment is recommended, with the addition of pain-modulating oral medication, such as a tricyclic antidepressant or gabapentin, in unresponsive cases.

Penile Dysaesthesia Men may develop a similar problem, with an abnormal burning sensation on the glans or around the urethral meatus. The management is as for female patients.

Psychosexual Problems

Men and women who have any chronic genital disorder will often lose their interest in sexual activity, leading to problems with sexual dysfunction.[54,55] It is important to give the patient the opportunity to express their concerns about their sexual function, and to offer a referral to someone with the necessary expertise to address these problems. Women are more likely to bring up sexual matters if they have seen the doctor before and feel comfortable with the consultation. However, many patients are too embarrassed to bring up the topic of sexual function and it is important that the doctor asks a simple question about sexual activity and associated concerns. Sometimes it is the patient's partner who has a problem and does not wish to have physical contact for fear of hurting the partner or 'catching' the disease.

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