British Association of Dermatologists’ guidelines for the management of lichen sclerosus 2010

Abstract

One of the aims of the British Association of Dermatologists (BAD) is to provide guidelines for the management of skin diseases using all available good‐quality evidence‐based data. The BAD guidelines writing and consultation process, and its revised formats, have been described elsewhere.1 2–3 These guidelines for the management of lichen sclerosus (LS) have been prepared for dermatologists on behalf of the BAD. They present evidence‐based guidance for investigation and treatment, with identification of the strength of evidence available at the time of preparation of the guidelines. The guidelines have been revised and updated in accordance with a predetermined scope, based on that used in the 2002 guidelines. Recommendations in these guidelines supersede those in the 2002 guidelines. The overall objective of the guidelines is to provide up‐to‐date recommendations for the management of LS in adults and children. This guidance has been written by dermatologists and has been shown to a patient. The guidelines have also been seen by a urologist, gynaecologist and genitourinary physician, all of whom are involved in the management of patients with LS. These guidelines have been developed using the BAD’s recommendations3 and also with reference to the AGREE (Appraisal of Guidelines Research and Evaluation) instrument.4 Medline and EMBASE databases were searched from 2002 to 2009 and full relevant papers obtained. The draft guidelines were made available for consultation and review by the BAD membership; the final document was peer reviewed by the Clinical Standards Unit of the BAD (made up of the Therapy & Guidelines and Audit & Clinical Standards Subcommittees) prior to publication. There are few published randomized controlled trials to support the following guidelines for the management of LS; the recommendations made are those that are currently considered best practice but they will be modified at intervals in the light of new evidence. LS, although a dermatosis, occurs commonly at a genital site and consequently is not only managed by dermatologists; patients may be under the care of other specialist disciplines. There have been many long‐standing difficulties in the appraisal and grading of the evidence for the treatment of LS. Historically, the nomenclature used to describe LS has been unclear. In addition, there may be difficulty in assessing the number of patients with active disease as, although asymptomatic, there is still ongoing activity as evidenced by scarring. There are also instances where the LS may be in remission but the patient still experiences symptoms due to a secondary sensory disorder, or additional irritant eczema. These guidelines will be revised as necessary to reflect changes in practice. These guidelines have been prepared for dermatologists on behalf of the BAD and reflect the best data available at the time the report was prepared. Caution should be exercised in interpreting the data; the results of future studies may require alteration of the conclusions or recommendations in this report. It may be necessary or even desirable to depart from the guidelines in the interests of specific patients and special circumstances. Just as adherence to guidelines may not constitute defence against a claim of negligence, so deviation from them should not necessarily be deemed negligent. LS is an autoimmune, inflammatory dermatosis, characterized by a lymphocytic response that has a predilection for the genital skin in both sexes, and an association with several other autoimmune diseases. The aetiology of LS is uncertain but there is mounting evidence to suggest that autoimmune mechanisms are involved in its pathogenesis;5 6–7 there is an increased incidence of tissue‐specific antibodies8 and associations with other autoimmune diseases in patients with LS,9, 10 as well as positive associations with HLA class II antigens.11 12–13 There is still controversy regarding the implication of Borrelia infection as an aetiological agent; although several studies have shown that this association does not occur in the U.S.A., some doubt still remains in Europe.14, 15 The presence of circulating extracellular matrix protein antibodies gives further support to an immune aetiopathology in female patients.16‘Balanitis xerotica obliterans’ is now viewed as a synonymous term describing LS of the penis, and ‘kraurosis vulvae’ is now recognized as LS of the vulva. The term ‘leucoplakia’ (meaning white plaque) is not a diagnostic entity and is descriptive only, as many conditions may present with white plaques. The term ‘lichen sclerosus et atrophicus’ has been abbreviated to ‘lichen sclerosus’ as some cases are associated with a hypertrophic, rather than atrophic, epithelium. There are instances when it can be difficult to differentiate between LS and lichen planus (LP) on the basis of the clinical and histological features; these cases appear to constitute an overlap syndrome, which is often associated with squamous cell hyperplasia and a poor response to ultrapotent topical corticosteroids. In the main, these guidelines are for classical LS with typical clinical and histological features. LS is a relatively common dermatosis, although the true incidence is unknown, and probably underestimated, in part due to the distribution of patients among different clinical specialities and to the fact that it may be asymptomatic. Genital LS in female subjects has two peak ages of presentation – in the prepubertal and postmenopausal years.17 Although childhood LS usually improves, there may be cases that persist into adulthood.18 There is also a bimodal onset in male subjects, with age peaks in young boys and in adult men.19 Readers are referred to several reviews on the clinical, histological and pathogenetic aspects of LS20–23 and to a historical review of the nomenclature and therapy.24 The typical lesions are porcelain‐white papules and plaques, often associated with areas of ecchymosis. Follicular delling may be prominent, and occasionally hyperkeratosis is a prominent feature. The characteristic sites are the interlabial sulci, labia minora, clitoral hood, clitoris, perineal body and perineum. Genital mucosal involvement does not occur, the vagina and cervix always being spared (which is in contrast to LP), although there may be involvement at the mucocutaneous junctions (the vestibule), which may result in introital narrowing. Perianal lesions occur in women in 30% of cases. There may be extension to the buttocks and genitocrural folds. LS can Koebnerize and may first arise in an episiotomy scar. Itch is the main symptom, but pain may be a consequence of erosions or fissures. However, LS may also be entirely asymptomatic and an incidental finding on examination. In those with itch, this is often worse at night and may be sufficiently severe to disturb sleep. Dyspareunia occurs in the presence of erosions, fissures or introital narrowing. LS is a scarring process and may cause loss of the labia minora, sealing of the clitoral hood and burying of the clitoris. Severe introital stenosis may rarely occur, but is seen more frequently in the LS/LP overlap syndrome. The lesions are similar to those in adult women, but ecchymosis may be very striking and potentially mistaken as evidence of sexual abuse.25 The confirmation of a diagnosis of LS does not, however, totally exclude coincident sexual abuse as some cases of LS may possibly be caused or aggravated by sexual abuse through Koebnerization.26 Features that should arouse suspicion of this include LS arising in older prepubertal girls, poor response to treatment, the presence of associated sexually transmitted infection or other symptoms or signs of abuse. Milia may also be present transiently in treated and untreated disease. Perianal involvement is a frequent finding in young girls, who may present with constipation because of painful fissuring in this area. The common sites of involvement of LS in adult men are the prepuce, coronal sulcus and glans penis, and more rarely lesions may be found on the shaft of the penis. The presenting complaint is usually tightening of the foreskin, which may lead to phimosis. This in turn results in erectile dysfunction and painful erections. One report documents that 30% of phimosis occurring in adults was due to LS,27 although another study of 75 subjects with severe phimosis identified LS in only 11%.28 Other presenting complaints are due to the appearance of lesions or changes in urinary stream, but itch is not a prominent symptom. Perianal disease is rarely, if ever, seen in male patients. The perimeatal area may be involved and postinflammatory scarring may lead to stenosis and obstruction. There may also be more proximal urethral involvement although this usually starts at the meatus.23 These complications may require a multidisciplinary approach with input from both a dermatologist and urologist. The disease usually affects the prepuce and the most frequent presentation is phimosis. The reported incidence of LS in children with phimosis ranges from 14% to 100%.29 30–31 Perianal involvement, as in adult men, is extremely rare. There is a report of a rare complication of renal failure following meatal obstruction.32 The classical sites for extragenital lesions are the upper trunk, axillae, buttocks and lateral thighs, and these are involved most frequently in adult women. Rarer sites include the mouth, face, scalp, hands, feet and nails. The typical lesions are porcelain‐white plaques, which may have follicular dells and areas of ecchymosis, similar to the genital lesions. In extragenital sites, there may be difficulty in distinguishing the lesions from those of morphoea. The clinical types of extragenital LS include an extensive bullous form,33, 34 as well as annular, Blaschkoid and keratotic variants.35 Koebnerization is very common at extragenital sites, arising at pressure points, old surgical and radiotherapy scars and at sites of trauma. A confirmatory biopsy, although ideal, is not always practical, particularly in children. It is not always essential when the clinical features are typical. However, histological examination is advisable if there are atypical features or diagnostic uncertainty and is mandatory if there is any suspicion of neoplastic change. Patients under routine follow‐up will need a biopsy if: (i) there is a suspicion of neoplastic change, i.e. a persistent area of hyperkeratosis, erosion or erythema, or new warty or papular lesions; (ii) the disease fails to respond to adequate treatment; (iii) there is extragenital LS, with features suggesting an overlap with morphoea; (iv) there are pigmented areas, in order to exclude an abnormal melanocytic proliferation; and (v) second‐line therapy is to be used. An autoantibody screen to look for associated autoimmune disease is useful if there are clinical features to suggest an autoimmune disorder. In particular, thyroid disease is common in women with LS.36 Swabs are not required routinely but may be indicated in erosive disease to exclude herpes simplex or Candida as additional complicating problems.37 Retesting for these infections may be necessary in disease that flares or fails to respond to treatment. If there is an abnormal vaginal discharge this will need appropriate investigation. 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 of these There appear to be two pathogenetic mechanisms for vulval in women is associated with the and in older women, the association is with a scarring as LS or with if evidence of a with arising LS only occurs in disease. The is being However, examination of vulval that occur on a of LS may as both an and of by mechanisms that to be of Although there is evidence for an for in there has been a that topical may may be found in vulval associated with of the should be managed by in this as has to be to the and particularly in disease. An association between LS and penile has also been Although histological evidence of LS can be found in of penile carcinoma the of this complication in any patient with LS is data suggest that the is similar to the for female In a of male patients with genital LS, histological changes of were found in verrucous carcinoma in two and of in The of in penile has also been studies using have a of in but other studies have a of up to An additional that has been with penile is the of a prominent on LS, suggesting disease This is if and or difficulty with may need to be of the vaginal is used in the to further and stenosis due to clitoral hood the and up a painful This a or If or between the of the prepuce and the glans persist adequate treatment, these will need to be treated and a at the phimosis will also require a If the disease is still active at the time of a topical be required to Koebnerization and further particularly the coronal If this results in an urinary stream, for is These conditions may occur an inflammatory of the or the patient remains objective clinical or of the skin lesions. pain does not respond to topical and treatment be to the of the is with the of as a or in cases. may a similar with an abnormal on the glans or the urethral The management is as for female patients. and women who have any genital will often in sexual to with sexual It is to the patient the to sexual and to a to with the necessary to these are more to up sexual if they have seen the and with the However, many patients are to up the of sexual and it is that the a sexual activity and associated it is the who has a and does not to have for of the or the disease. have the of treatment for LS. There are no randomized controlled trials evidence that a or of any specific is the most or that is to However, the and treatment is the very topical of of evidence for The by the for a is at for on for and for a further The for is based on studies that an ultrapotent only to be a on extragenital If symptoms when the of is the patient is to the treatment more often the symptoms They can to the A of should at If the treatment has been the hyperkeratosis, fissuring and erosions should have but the scarring and its associated will of patients remission of will to have flares and they are to as patients with ongoing disease to require of In the of in this is and there has been no evidence of or an in the incidence of There is study of up to the of One study using the less that this was also A study of 34 postmenopausal women with vulval LS that the of an in to the topical the treatment and as is very patients of scarring A is also An on LS, with the for the of the topical should be to the patient. A study of men treated with this to be and with in and skin and also in urinary in the patients in whom this was The of topical in men may also the need for of of evidence There are no ongoing randomized controlled trials to the of a topical as being the treatment of for childhood LS in In a of cases of childhood vulval LS, topical were treatment to support this A by boys with phimosis using for that of the this to as treatment and required ongoing topical One using a in disease with Other studies have shown that phimosis may with the of an ultrapotent or topical a need for in a of boys with only of whom LS, to for but only of the patients with LS this that phimosis due to other than LS may also respond to topical of of evidence There are no randomized controlled trials on which to recommendations but with or is used as and when In extragenital lesions are not as as genital disease to topical Although it has been used in the there to be no evidence for the of topical There is a report of the of topical Although LS predominantly affects the genital in female suggesting a therapy to have any on the is no used. There is no evidence for the of topical or in children. There is no for of vulval in the management of LS, and should be used for malignancy and postinflammatory In study of patients with vulval LS and severe itch, with for of of evidence In an study of therapy for vulval LS to 10 of patients of of evidence study in of 10 patients treated with using a A study of in women with vulval LS that not been controlled by topical reported in although two and the required ongoing treatment with topical of of evidence treatment, in a study of 10 was but not the disease It was in patient with urethral of of evidence There is a report of the of in of cases of untreated LS using of of of evidence Although the treatment for male patients with LS is a topical it is not always when scarring has to The of for penile LS with symptoms due to persistent phimosis or meatal stenosis is with studies In a of men with penile LS, and follow‐up of in 34 was in in and in and of in LS is rare in the but does not always against further flares of the disease. One that of men to have lesions of and that the LS may Koebnerize in the scar. Koebnerization may be the for the of urethral which is seen more frequently in patients with LS. This complication to be most common in those a rather than with a of of evidence treatment has been used to meatal but this is not practice. One study of men with LS results treatment no evidence of of of evidence treatment is urethral or A topical may be required at the time as treatment of childhood phimosis by has the presence of LS in a of cases. It is now being recognized that a of a topical should be prior to in all cases of phimosis of aetiology and that should be for treatment of of evidence has been and has been reported to in symptoms and appearance of lesions. of have been used for extragenital LS, or with topical and The to be the most in clinical as well as of these have only been reported as cases or One study the of on two areas of extragenital LS in patient. The site treated with the a response than the of of evidence The of topical and has been in women with vulval LS, and A study of patients women, men and has the of A study on the of in prepubertal also an in However, on was often the of these is not and there are an increased of with in a disease with a There are of in patients who have been using these and studies are of in LS. It is that the should not be used as treatment of of evidence A of topical to have any clinical or histological in cases of vulval However, was reported as in symptoms and erosions in a of patients with has been used with in an of extragenital and may also be an for of of evidence There is a study of the of and which an of of evidence topical and have been used to There is no evidence that these are particularly in LS. there is some evidence that they may have a in and disease that does not respond to an ultrapotent One report of patients with LS at sites, and to other with in all ranges from to in of of evidence There are of from and as and which the main is the uncertain with Borrelia These and are in reviews but all be viewed as less or If treatment with topical to to LS under it is to the an patients may be at the of the against the of topical in the area. patients with poor and may not be to the the diagnosis been made or is there an additional as the of a to the urinary herpes simplex or there a secondary sensory the LS been but the patient remains because a secondary sensory has or are there with which the to there a due to as severe phimosis or meatal stenosis in in which may be The of malignancy in genital LS that has been and treated is very If malignancy occurs it to The suggest two follow‐up the at to response to treatment and to that the patient is using the topical and and a final to that the patient is in and to the to any that the patient have discharge to the care of If patients to a topical it is that they care a should be to the patient at the time of discharge from the them that any persistent area of erythema, or new be reported to who will an to an appropriate However, as of women from vulval are not up in care it is that for are follow‐up in a secondary care specialist is appropriate for patients with genital LS associated with skin or or uncertainty The is for male patients with this of persistent erosions, hyperkeratosis and areas are to exclude or These patients usually have LS with a histological that has features of both LS and with squamous cell these patients to have an overlap and disease a trials of and a on to or more An ultrapotent topical is the treatment for LS in or age at any but there are no randomized controlled trials of and of treatment. patients with evidence of active LS hyperkeratosis and should be LS is associated with but the of this complication is rare in clinical It is not treatment the of change. follow‐up in a is for disease that is using of a topical i.e. in care follow‐up should be for patients with LS that is to treatment and those patients who have persistent disease with a of a A should be in any patient with atypical or controlled LS. is only indicated for the complications of or an in female patients. It may be useful in male patients with severe phimosis. If these should be if made to a in this a biopsy been in patients with active LS that has not to follow‐up in for patients with ongoing patients with genital LS of the need to report any lesions the a topical of adequate and been used prior to in with always reported on male The to the made by the to the preparation of both the guidelines and this to and the care of patients be was a very and is a loss to of evidence with a of evidence should not be used as a basis for a randomized controlled of randomized controlled for and Clinical of This is an updated prepared for the British Association of (BAD) Clinical Standards made up of the Therapy & Guidelines and the Audit & Clinical Standards of the Clinical Standards Unit and Clinical Standards Guidelines in 2002 by the British Association of reviewed and updated has the process used by the British Association of Dermatologists to guidelines. is for from and is to guidance using the described in the British Association of guidelines & on can be viewed at