Lichen Sclerosus 2002
Lichen Sclerosus 2002
Lichen Sclerosus 2002
GUIDELINES
Guidelines for the management of lichen sclerosus
S.M.NEILL, F.M.TATNALL* AND N.H.COX†
St Peter’s Hospital, Chertsey, Surrey, St John’s Dermatology Centre, St Thomas’ Hospital, London and Chelsea and Westminster
Hospital, London, U.K.
*Watford General Hospital, Vicarage Road, Watford WD1 8HB, U.K.
†Cumberland Infirmary, Carlisle CA2 7HY, U.K.
Summary These guidelines for the management of lichen sclerosus have been prepared for dermatologists on
behalf of the British Association of Dermatologists. They present evidence-based guidance for
treatment, with identification of the strength of evidence available at the time of preparation of the
guidelines, and a brief overview of epidemiological aspects, diagnosis and investigation.
Key words: guidelines, lichen sclerosus
Introduction Definition
The aim of the British Association of Dermatologists is to LS is a lymphocyte-mediated dermatosis that has a
provide guidelines for the management of skin diseases predilection for the genital skin in both sexes and was
first formally described at the end of the nineteenth
Correspondence: Dr N.H.Cox. century by Hallopeau and Darier as a variant of lichen
E-mail: [email protected] planus (LP).1–3 Use of the terms leucoplakia and
These guidelines were prepared for the British Association of Der- kraurosis vulvae4 in older literature is a source of
matologists Therapy Guidelines and Audit subcommittee. Members of confusion. Balanitis xerotica obliterans, a term used for
the committee are: N.H.Cox (Chairman), A.V.Anstey, C.B.Bunker,
LS of the penis, was only recently coded as part of LS in
M.J.D.Goodfield, A.S.Highet, D.Mehta, R.H.Meyrick Thomas, A.D.
Ormerod, J.K.Schofield and C.H.Smith. These guidelines have been literature search tools such as Medline. Not all LS is
endorsed by the Clinical Effectiveness Group of the Association for histologically atrophic, and the term lichen sclerosus et
Genitourinary Medicine and the Medical Society for the Study of atrophicus has now been replaced with LS alone.
Venereal Diseases (Chair: Dr K.W.Radcliffe). We are grateful for
Currently, LS is considered as a separate entity from
additional input from the Royal College of Obstetricians and Gynae-
cologists. LP on the basis of its specific distinguishing clinical and
Conflict of interest: none. histological features. However, it is recognized that
some cases of LS may represent an overlap syndrome, interlabial sulci, labia minora, clitoral hood, clitoris and
sharing features of both LS and LP, and perhaps the perineal body. Genital mucosal involvement does not
two conditions represent different parts of a spectrum of occur, the vagina and cervix always being spared (by
the same process. Such overlap cases are often associ- contrast with LP). However, there may be some
ated histologically with squamous cell hyperplasia, and mucosal involvement at the edge of mucocutaneous
are best categorized as complicated LS, as response to junctions, which may lead to introital narrowing.
a topical ultrapotent corticosteroid is often poorer.5 Perianal lesions occur in women in 30% of cases. Itch
These guidelines are in the main for uncomplicated is the main symptom but pain occurs if there are
LS with the classical histological features of the erosions or fissures. The itch is worse at nights and
disorder. may be so severe as to disturb sleep. Dyspareunia
occurs in the presence of erosions, fissures or introital
narrowing.
Pathogenesis
Some women are asymptomatic and the LS is only
The aetiology of LS is uncertain but there is mounting discovered when they are being examined for another
evidence to suggest that autoimmune mechanisms are reason. Most of these patients have inactive disease,
involved in its pathogenesis;6–8 there is an increased which may have occurred in childhood, and the
incidence of tissue-specific antibodies9 and associations changes seen are the long-standing atrophic changes
with other autoimmune diseases in patients with that persist. However, some of these individuals have
LS,10,11 as well as positive associations with HLA class changes of active disease with hyperkeratosis and
II antigens.12–14 There is still controversy regarding the ecchymosis, and should therefore receive treatment.
implication of Borrelia infection as an aetiological
agent; although several studies have shown that this
Female anogenital: child
association does not occur in the U.S.A., some doubt
still remains in Europe.15,16 The lesions are similar to those in adult women but
ecchymosis may be very striking and potentially
mistaken as evidence of sexual abuse.20 There has
Incidence
been a tendency to exclude a diagnosis of sexual abuse
It is clear from the number of patients attending a if LS has been confirmed; however, as LS exhibits the
vulval clinic that LS is a common disease. LS in females Koebner phenomenon at sites of trauma, some cases of
has two peak ages of presentation. The first of these LS may in fact be caused or aggravated by sexual
occurs in prepubertal girls17 and may resolve or abuse.21 Suspicious features include LS in older prepu-
continue beyond the menarche.18 The other peak of bertal girls, the presence of associated infection (espe-
incidence is in postmenopausal women;17 although cially infections that are characteristically sexually
this suggests a hormonal influence, hormone replace- transmitted), or other symptoms or signs of abuse.
ment therapy neither improves existing disease nor Perianal LS with or without vulval involvement
provides any protection against its development. Inter- occurs in young girls, who commonly present with
estingly, pregnancy seems to improve the symptoms constipation because of painful fissuring in this area.
and signs, and a normal vaginal delivery is usually
possible.
Male genital: adult
The incidence of LS in males is lower than in females
but there is also a bimodal onset, with peaks of disease The lesions appear most commonly on the prepuce,
presentation occurring in young boys and then again coronal sulcus and glans penis. More rarely lesions
in adults.19 may be found on the shaft of the penis. The presenting
complaint is usually tightening of the foreskin, which
may lead to phimosis and painful erections. One report
Clinical features documented that 40% of phimosis occurring in adults
was due to LS,22 although another study of 75 subjects
Female anogenital: adult
with severe phimosis only identified LS in eight
The typical lesions are porcelain-white papules and (11%).23 In contrast to women, men commonly
plaques, often with areas of ecchymosis. Follicular present with the consequences of scarring. The perim-
delling may be prominent. The lesions occur in the eatal area may be involved and postinflammatory
Extragenital
Complications
The classical extragenital sites are the upper trunk,
Malignancy
axillae, buttocks and lateral thighs. Extragenital
lesions in men are uncommon and usually do not SCC, discussed below, is the commonest malignancy
occur in association with genital lesions. The face and described in association with anogenital LS. Interest-
scalp in both sexes are other sites that may rarely be ingly, SCC has not been recorded in LS at sites other
involved. than the anogenital area. Verrucous carcinomas also
Oral lesions of LS are extremely rare and many of the appear to occur on a background of LS.29,30 There have
cases reported in the literature have not been substan- also been reports of basal cell carcinoma31 and
tiated with histological evidence and may well have melanoma occurring in cases of vulval LS.32,33
been examples of LP or morphoea.26 In the rare
instances of oral LS these are recorded at sites in the Squamous cell carcinoma in women with genital lichen
mouth where there is cornified stratified squamous sclerosus. SCC arising within LS only occurs in lesions
epithelium, i.e. tongue, gingiva and hard palate. affecting the anogenital area; in practice, this risk is
extremely small. Two studies each of over 200 women
with LS under regular review have shown a small but
Investigations
definite increased incidence of invasive SCC.17,34 The
The diagnosis in most patients is usually made clinic- magnitude of this risk is about 5% or less lifelong in
ally, but a confirmatory biopsy is helpful in cases where known patients with LS17,30 (and is therefore probably
there is some clinical doubt about the diagnosis and to a significant overestimate in view of the likely high
document any atypical features. The main differential prevalence of undiagnosed cases of LS). However,
diagnoses include LP, mucous membrane pemphigoid histopathological examination of vulval SCCs indicates
and genital psoriasis. A skin biopsy is not always that about 60% occur on a background of LS.35–38 A
practical in children and it is preferable to initiate their clinical study of anogenital SCC presenting to a vulval
treatment without histological confirmation. A biopsy clinic demonstrated that 14 of 23 cases occurred on a
is essential in all cases that fail to respond to adequate background of LS.30 The role of human papilloma-
treatment. virus (HPV) as a possible aetiological agent in the
progression to malignancy in LS has not been clearly Pseudocyst of the clitoris. Occasionally, clitoral hood
established, although recent evidence suggests that adhesions seal over the clitoris and keratinous debris
there may be two distinct aetiologies for vulval SCC. builds up underneath, forming a painful pseudocyst.
One type occurs in older women with a chronic This requires a subtotal or total circumcision.50
dermatosis such as LS, the other in younger women
without LS but with evidence of the same oncogenic Phimosis. The most common complication in males is
HPV types that are linked to cervical SCC.39 Although secondary phimosis, which may require circumcision if
evidence for an important role of HPV in LS-associated medical treatment fails. If the disease is still active at
SCC is scanty, there is a remote theoretical risk that the time of surgery it is important to continue topical
topical corticosteroid use might induce oncogenic HPV corticosteroids following the surgery to prevent Koeb-
types that may be cause for concern because, as found nerization and further scarring, particularly around the
in the normal population, up to 20% of cases of LS may coronal sulcus.
incidentally carry the oncogenic HPV 16.40
SCC of the vulva should be managed by gynaecol- Meatal stenosis. LS of the glans may cause meatal
ogists experienced in this field as surgery has to be stenosis, which is manifest as an altered urinary
individualized according to the tumour size and loca- stream, less commonly progressing to cause frank
tion, particularly in early invasive disease.41 obstruction to urinary flow.
Topical progesterone has also been reported to be Extragenital lichen sclerosus. Shave (tangential) excision
effective69 (Strength of evidence C, Quality of evidence IV). has been used,78 and carbon dioxide laser has been
reported to produce an improvement in symptoms and
Male genital lichen sclerosus. Testosterone has also appearance of lesions.76
been used topically (2Æ5% ointment) for male genital A case of extragenital LS in a child has been
LS70 (Strength of recommendation C, Quality of evidence successfully treated with low-dose ultraviolet (UV) A1
IV). phototherapy.79
preparation. Also, very elderly patients disabled with runs a relentless course despite trials of various
poor eyesight and limited mobility may not be able to therapies, and a small percentage does go on to develop
apply the medication appropriately. one or more SCCs.
2 Is the diagnosis correct, or is there an added problem It is important to biopsy persistent ulcers, erosions,
such as the development of a contact allergy to the hyperkeratosis and erythematous zones, whether pre-
medication or is there another superimposed condition, sent at initial presentation or subsequently, to exclude
e.g. secondary candidiasis, intraepithelial neoplasia, intraepithelial neoplasia or invasive SCC.
malignancy, psoriasis or mucous membrane pemphig-
oid?
Recommendations and conclusions
3 Is the LS in fact treated, but the patient is still
symptomatic because they have developed a secon- An ultrapotent topical corticosteroid is the first-line
dary sensory problem, dysaesthetic vulvodynia or are treatment for LS in either sex at any site, but there are
experiencing problems with intercourse that they may no randomized controlled trials comparing corticoster-
feel too shy to discuss? oid potency, frequency of application and duration of
4 Is the problem mechanical due to scarring, e.g. treatment.
severe phimosis or meatal stenosis in males, in which Asymptomatic patients with evidence of clinically
case surgery may be indicated? active LS, i.e. ecchymosis, hyperkeratosis and progress-
ing atrophy, should be treated.
Anogenital LS is associated with SCC but the
Follow-up
development of this complication is rare in clinical
The risk of malignancy in uncomplicated genital LS practice (5% or less). It is not yet known whether
that has been diagnosed and treated appropriately is treatment will lessen the long-term risk of malignant
very small. If malignancy occurs it does so rapidly. change.
Early detection would require 3-monthly follow-up Long-term follow up in a specialized clinic is unnec-
consultations; this is generally impossible in the U.K. essary for uncomplicated disease that is well controlled
due to the constraints of the National Health Service clinically using small amounts of a topical corticoster-
system. oid, and follow up should be reserved for patients with
The authors suggest two follow-up visits after the complicated LS that is unresponsive to treatment and
initial consultation: (i) at 3 months to assess response those patients who have persistent disease with history
to treatment and to ensure that the patient is using the of a previous SCC.
topical corticosteroid appropriately and judiciously, Surgical intervention is indicated only for the com-
and (ii) if response has been satisfactory, a final plications of scarring or the development of malignancy.
assessment 6 months later to ensure that the patient Any psychosexual issues should be addressed if
is confident in treating their problem and to take the appropriate and referral made to practitioners experi-
opportunity to discuss any residual problems that the enced in this field if indicated.
patient might have before discharge back to the care of
their primary physician. If patients continue to use a
Audit points
topical corticosteroid it is suggested that they see their
primary care physician once yearly. Instruction should • Has a biopsy been performed in patients with
be given to the patient at the time of their discharge clinically active disease that is unresponsive to ad-
from the clinic warning them that any persistent equate treatment with an ultrapotent topical cortico-
ulceration or new growth must be reported to their steroid?
family practitioner who will then make an urgent • Are follow-up arrangements in place for patients
referral back to an appropriate specialist. with ongoing symptomatic disease?
Long-term follow up is, however, required for • Are patients with genital LS aware that any persist-
patients with LS that continues to be poorly controlled. ent ulcer, erosion or new growth within the affected
These patients usually have LS with a histological skin needs to be reported?
pattern that has features of both LS and LP with • Has a topical corticosteroid of adequate potency and
squamous cell hyperplasia. Clinically, these patients duration been used prior to surgery in males with
seem to have an overlap syndrome and their disease symptomatic preputial tightening?
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