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Diagnosis and Treatment of Vulvar Lichen Sclerosus: An Update for Dermatologists

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Diagnosis and Treatment of Vulvar Lichen Sclerosus: An Update for Dermatologists

Abstract

Vulvar lichen sclerosus is an important skin disease that is common in women in their 50 s and beyond; however, it can also affect females of any age, including children. If not treated, it has the potential to cause significant and permanent scarring and deformity of the vulvar structure. In addition, if untreated, it is associated with a 2–6% lifetime risk of malignant squamous neoplasia of the vulva. Lichen sclerosus has been considered a difficult to manage condition; however, both serious complications can potentially be prevented with early intervention with topical corticosteroid, suggesting that the course of the disease can be treatment modified.
1 23
American Journal of Clinical
Dermatology
ISSN 1175-0561
Am J Clin Dermatol
DOI 10.1007/s40257-018-0364-7
Diagnosis and Treatment of Vulvar Lichen
Sclerosus: An Update for Dermatologists
Andrew Lee & Gayle Fischer
1 23
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Vol.:(0123456789)
American Journal of Clinical Dermatology
https://doi.org/10.1007/s40257-018-0364-7
REVIEW ARTICLE
Diagnosis andTreatment ofVulvar Lichen Sclerosus: AnUpdate
forDermatologists
AndrewLee1,2,3· GayleFischer1,2,3
© Springer International Publishing AG, part of Springer Nature 2018
Abstract
Vulvar lichen sclerosus is an important skin disease that is common in women in their 50s and beyond; however, it can also
affect females of any age, including children. If not treated, it has the potential to cause significant and permanent scarring
and deformity of the vulvar structure. In addition, if untreated, it is associated with a 2–6% lifetime risk of malignant squa-
mous neoplasia of the vulva. Lichen sclerosus has been considered a difficult to manage condition; however, both serious
complications can potentially be prevented with early intervention with topical corticosteroid, suggesting that the course of
the disease can be treatment modified.
Key Points
Vulvar lichen sclerosus treated inadequately can lead to
scarring and permanent deformity of the vulva.
Long-term maintenance treatment with topical cortico-
steroids can keep vulvar lichen sclerosus in remission
and potentially reduce the risk of vulvar malignancy.
Patients with vulvar lichen sclerosus require long-term
follow-up even after initial remission is achieved, with
a physician who is adequately experienced in managing
this condition, as it may continue to silently progress or
reappear without causing symptoms.
1 Introduction
Lichen sclerosus (LS) is an uncommon and potentially seri-
ous skin disease that has a predilection for the genital skin
and is much more often encountered in females than males.
This makes it common in vulvar practice, responsible for at
least 10% of new cases [1]. The prevalence in the whole pop-
ulation is essentially unknown because of under-diagnosis
and referral bias. It has been reported as 1.7% of all patients
in a general gynecology practice [2]. A recent study from
the Netherlands estimated the incidence at 14.6 per 100,000
women years [3]. It occurs in all age groups, including chil-
dren. The mean age of onset is in the mid to late 50s, with
only about one third of cases occurring in women under
50years [2, 3]. In the pediatric group, vulvar lichen sclero-
sus (VLS) can occur as early as in the first few years of life.
Pediatric disease accounts for 7–15% of all cases [4]. In the
pediatric setting, LS almost always affects the genital area,
with only approximately 6% of these patients having extra-
genitalinvolvement [5]. Until recently, it was believed that
pre-pubertal VLS resolved at puberty, but recent evidence
has shown that this is not consistently true [6, 7].
Although LS may occur on any part of the skin, it is
almost always a genital condition, involving the vulva, peri-
neum and perianal skin. Extragenital lesions are most com-
mon on the neck, buttocks, inner thigh, shoulders and wrists
(Fig.1).
VLS is an important condition to diagnose correctly
and manage actively for two reasons. First, if not treated
aggressively, it may significantly scar, shrink and deform
* Gayle Fischer
gayle.fischer@sydney.edu.au
1 Department ofDermatology, Royal North Shore Hospital,
Reserve Road, StLeonards, NSW2065, Australia
2 Sydney Medical School Northern, The University ofSydney,
StLeonards, NSW, Australia
3 Department ofDermatology, Kolling Institute, Northern
Sydney Local Health District, StLeonards, NSW, Australia
Author's personal copy
A.Lee, G.Fischer
the vulva and cause stenosis of the introitus, with result-
ing impact on quality of life. Second, it is a risk factor for
malignant squamous cell neoplasia of the vulva, including
invasive squamous cell carcinoma and vulvar intraepithelial
neoplasia, with thelifetime risk of untreated or inadequately
treated disease being 2–6% [3, 8]. This is unusual for a skin
condition. Rarely do either of these complications occur in
other dermatoses, an example being cutaneous discoid lupus.
However, recent research suggests that both can be prevented
by early intervention, and even with later intervention, can-
cer and further scarring can be arrested [9]. Therefore, these
patients require lifelongobservation and encouragement
to continue treatment. This is a fact that has been under-
investigated and is therefore lacking in much of the existing
literature on the subject.
2 Etiology
The true etiology of LS remains unknown. Studies have
observed high rates of autoimmune disease in patients with
LS and even higher levels of detection of autoantibodies, but
this does not confirm that LS itself is definitely autoimmune.
A confounding factor is that middle-aged female patients,
the main cohort with this condition, have a relatively high
rate of autoantibodies. A well conducted study found that
relative to aged-matched controls, LS patients do suffer from
autoimmune disease more often: about 30% as opposed to
10% in the whole population [10]. As well, about 30% had
a positive family history. However, the same study found
that there was no significant difference between the rate of
autoantibody detection between patients with LS and con-
trols. The two diseases seen most often in association were
autoimmune thyroid disease and vitiligo.
Additionally morphoea, alopecia areata and pernicious
anemia have been linked to LS [11, 12]. Diabetes, psoriasis
and coeliac disease have all been reported to co-exist, but it
is possible that this is coincidence [1315]. More recently,
LS has been found to have a higher incidence (17%) in
patients with Turner’s syndrome [16].
A study has demonstrated that 67% of patients in a cohort
of 30 with LS had serum immunoglobulin G (IgG) antibod-
ies to ECM-1 compared to 7% of controls [17]. This often
quoted research unfortunately has not brought us any closer
to knowing the pathogenesis; however, it does strengthen the
presumption that VLS is an autoimmune condition despite
the fact that this has never been confirmed.
The majority of patients with LS are otherwise well with
no personal or family history of autoimmune disease. It is
not uncommon to find low titer positive antinuclear antibody
(ANA); however, thisis rarely significant enough to war-
rant further investigation. Thyroidautoantibodies may also
be present, and if this is the case, further investigation is
warranted. Even in the presence of thyroid auto-antibodies,
thyroid function may be normal [18].
LS can certainly run in families, although it is more com-
mon for it to be a chance phenomenon [19]. This has led to
a search for a human leukocyte antigen (HLA) association.
Although no association with the autoimmune-related HLA
antigens (HLA A1, B8 and DR3) has been reported, the
HLA class II antigens HLA-DQ7, HLA-DR11 and HLA-
DR12 have the strongest association with susceptibility to
LS [20].While these documented HLA associations are of
interest, unfortunately, there is a lack of significant data to
conclusively comment on the strength of these associations.
Epigenetic changes can cause functional impairment of
the genome not related to DNA sequence. These changes
can lead to altered gene expression and phenotypic changes.
Recent research has identified altered enzyme expression in
VLS resulting from an epigenetic change and pointing to a
possible epigenetic background for pathogenesis [21].
3 Diagnosis andDisease Course
3.1 Clinical Presentation inAdults
The most common presenting symptom of VLS is itch
(93%), often of a severe, life- and sleep-disrupting nature.
There is sometimes pain as a result of excoriation or fis-
suring [9]. Distressing clitoral hyperesthesia may occur,
and dyspareunia is very common. Other symptoms include
Fig. 1 Extragenital lichen sclerosuson the back
Author's personal copy
Vuvlar Lichen Sclerosus: An Update
dysuria, sexual dysfunction and bleeding from skin fis-
sures of the vulva and perianal skin.
VLS has a significant impact on quality of life and
sexual functioning [22]. However, occasionally, VLS
can be completely asymptomatic, discovered by chance
by the patient or by the general practitioner during a Pap
smear test. This is rare but important, as the disease may
be advanced at presentation as a result. This situation can
be dangerous, as asymptomatic disease may not be noticed
by the patient until carcinoma arises [23].
The appearance of a well-defined white sclerotic plaque
with an atrophic wrinkled surface is typical (Fig.2). How-
ever, there are many variations. These include:
Multiple white papules or macules producing a speckled
appearance
Thickened hyperkeratotic plaques
Plaques limited to small areas such as the tips of the labia
minora or the clitoris or clitoral hood
Edema on a background of pallor
Telangiectasia, purpura, hemorrhagic blistering on a
background of pallor
Angiokeratomas on a background of pallor (Fig.3)
Fissures and traumatic ulcers (Fig.4)
Erosions
Blisters
LS associated with vulvar psoriasis, which appearsery-
thematous
Brown hyperpigmentation similar to post-inflammatory
hyperpigmentation which cansupervene (Fig.5).
The distribution of VLS is also very variable. The clas-
sic textbook description is of a figure of eight encircling the
vulva, perineum and perianal skin. However, it can affect
only the perianal region, clitoris, and internal surface of the
labia majora, labia minora, and the vaginal introitus. VLS
very rarely involves the vagina proper (that is, within the
hymen). The exception is where squamatization of the vagi-
nal wall has occurred as a result of prolapse [24].
A key point in recognizing VLS, particularly in the late
stage, is that the vulvar shape is often not normal. While it
is true that the size of the labia minora is highly variable,
virtually all women develop them. If they are completely
Fig. 2 Lichen sclerosuswith a typical atrophic wrinkled surface
Fig. 3 Angiokeratomas on a background of pallor(lichen sclerosus)
Fig. 4 Lichen sclerosus: fissures and traumatic erosions
Author's personal copy
A.Lee, G.Fischer
missing or if the clitoris has shrunk or is buried under scar
tissue, this is very suggestive of this condition, and indeed
the only other conditions which can result in this appear-
ance are lichen planus, graft-versus-host disease and the
very rare mucous membrane pemphigoid, all of which are
erosive conditions. In some cases, VLS and lichen planus
can occur concurrently in an overlap condition, with VLS
of the vulvar skin and lichen planus involving the vestibule
and vagina [25].
VLS obeys the Koebner phenomenon, and therefore
localizes into areas of friction and trauma. This possibly
explains why it is usually most recalcitrant on the perineum
and the inner surfaces of the labia minora. It has also raised
a question regarding the role of urinary incontinence, par-
ticularly in the setting of treatment resistance [26].
3.2 Dermoscopy
The dermoscopic appearance of VLS has recently been
documented. Patchy white structureless areas, comedo-like
openings, purpuric globules, scale, “ice slivers” and sparse,
thin vessels are described and are changed by treatment [27].
3.3 The Course oftheDisease inAdults
The course of VLS is unpredictable, but if left untreated,
about half of the patients will lose structure of the vulva,
with the labia minora eventually becoming reabsorbed, and
theclitoris becomes entrapped and buried, revealing an over-
all atrophic, shiny, white vulva missing normal anatomy [9].
The timeframe over which this happens is, anecdotally, a
few years, but has never been accurately estimated. It is very
typical for the labia minora to fuse with the labia majora
laterally and with each other subclitorally. The fusion line
is brittle and easily tears during intercourse. Perineal fis-
suring and tearing is also common. Eventually, the vaginal
opening may become significantly stenosed, with pooling
of urine within the vagina, simulating urinary incontinence.
Once scarring has occurred, it is in most cases irreversible.
In advanced disease, gross distortion of vulvar anatomy may
occur. There have not been any studies to determine which
patients are prone to scarring.
Between 2 and 6% of patients with VLS will progress to
malignancy [3, 28]. Which patients are prone to this is not
predictable, and it is therefore necessary to regard all as at
risk [29].
One study suggested that the patients with hyperkeratotic
disease were most at risk for malignant change, but this may
have been flawed by selection bias [30]. A more recent study
found that the only differentiating factor between those who
developed malignant change and those who did not was
whether their disease was consistently suppressed with treat-
ment [9].
In the past, there has been controversy regarding the
possibility that the course of the disease could be modi-
fied by treatment, and this has led to a nihilistic approach
that addresses only symptoms and not signs, which has
been expressed in the most recent guideline [31]. However,
a recent study has shown that ongoing topical corticosteroid
(TCS) maintenance treatment that is matched to the severity
of the disease so that it is suppressed will prevent both scar-
ring and carcinoma [9].
Little is known of the course of the disease in pregnancy;
however, recent data suggest that it is unchanged and that
treatment should be continued during pregnancy, without
adjustment from non-pregnant treatment. TCSs are consid-
ered safe in pregnancy. Patients with LS can usually have a
normal vaginal delivery [32].
3.4 Histopathology
The definitive diagnostic test for LS is a skin biopsy, which
should be taken from the most densely white area. The his-
topathology is distinctive and uniform across all ages. The
epidermis is often atrophic with hydropic degeneration of
basal cells and a homogenous pale zone of hyalinization in
the upper dermis. In the dermis, there is a variable lichenoid
infiltrate of mononuclear cells.
When VLS has been treated with TCS, the biopsy can
become non-specific; however, in this situation, the skin usu-
ally looks normal as well [33]. If a patient presents with a
presumptive diagnosis of VLS that is already treated, with
both normalcutaneous appearance and biopsy, the only
Fig. 5 Brown hyperpigmentation in lichen sclerosus, which appears
similar in appearance to post-inflammatory hyperpigmentation
Author's personal copy
Vuvlar Lichen Sclerosus: An Update
clue to the presence of the disease may therefore be loss of
structure.
Although VLS has a characteristic clinical appearance, a
skin biopsy, taken at first presentation, from the affected site
provides diagnostic confirmation and exclusion of alternate
diagnoses. A positive biopsy is also helpful in counseling
the patient about the important long-term consequences
and the need for follow-up. It is also useful if the patient
changes location or medical practitioners. Treated disease
may appear normal, and some authors have called for biopsy
prior to treatment whenever possible so that there is a clear,
histopathological record of the diagnosis [34].
In children, a clinical diagnosis is almost always suffi-
cient because biopsy is traumatic.The list of differential
diagnoses is small, including most frequently vitiligo and
lichenification, which may appear pale and is usually associ-
ated with eczema. Neoplastic transformation has never been
reported to occur in children with VLS.
Progression to carcinoma has not been reported in chil-
dren, although there have been several case reports of vul-
var melanoma in association with pediatric VLS [35]. Early
onset squamous malignancy has also been reported in adult
life when VLS first appeared in childhood [5].
3.5 Clinical Presentation inChildren
A recent study of 70 children with VLS showed the mean
age of development of symptoms was 5.0 years (range
1–12years) and the mean age at diagnosis was 6.7years
(range 3–14years) [5]. Another study of 46 children found
the mean age of diagnosis to be 7.8years, with a delay in
diagnosis of 1.6years [36]. Both studies indicate that many
children suffer for long periods of time before being diag-
nosed and treated. They also reported the most common
presenting symptoms were itching and soreness; however,
other symptoms or noted signs at presentation are purpura,
bleeding, dysuria, constipation, genital erosions andextra-
genital lesions. In one study, less than 10% of the children
studied were asymptomatic and were discovered after biopsy
for another reason [36]. Although children present with itch
and pain, about two thirds also report dysuria and pain with
defecation, leading to constipation [37]. These presentations
are quite different to adults, who normally present with itch
and dyspareunia. It is not uncommon for children with VLS
to be referred to urologists and gastroenterologists [37].
If purpura is present, children with VLS have been
reported to be referred to child protection units [38]. How-
ever, in VLS, purpura may occur without significant trauma,
and indeed, when purpura is caused by physical damage in
normal vulvar skin, it resolves quickly, unlike the purpura
of LS that is persistent (Fig.6).
The morphology of VLS in children does not differ from
adults. Older texts describe a figure of eight appearance
encircling the vulva and perianal skin, but any part of the
vulva, perineum and perianal skin may be affected individu-
ally. Scarring also occurs in children, and in rare circum-
stances, a child will present with loss of architecture with-
out the typical epithelial changes. Fissuring is common and
responsible for pain [36].
3.6 The Course oftheDisease inChildren
As in adults, if VLS is not treated in a child, progressive loss
of the vulvar architecture occurs [36]. The child may not
ever develop labia minora, and the clitoris may be buried
[25]. The clitoral hood may become non-retractile.
Although squamous malignancy has not been reported
in children, a study has shown that although human papil-
lomavirus (HPV) in children with VLS is not found more
frequently than in controls, when it is found, oncogenic HPV
genotypes are more common than in children without VLS,
and this raises questions about long-term cancer risk [39].
Older reports have suggested that LS in children will
resolve at or after puberty. However, this is not always the
case, and there are now studies that show that whilst there
may be improvement, true remission cannot be assumed [6].
One study has shown that at least 75% of girls still have
ongoing symptoms and signs [7].
As in adults, the course of the disease can be modified by
treatment [37]. A confounding problem in VLS is the ado-
lescent years. During this time, in the authors’ experience,
Fig. 6 Lichen sclerosus in a child characterized by pallor, purpura
and erosions
Author's personal copy
A.Lee, G.Fischer
compliance issues often arise linked to embarrassment and
refusal to be examined or aided by a parent. Even the most
trusting doctor–patient relationship may flounder at this
time.
Further, compliance can be a problem in managing chil-
dren when parents are unwilling or unable to supervise.
When treatment is left entirely to small children, it is under-
standable that it may be sub-optimally applied.
4 Dierential Diagnosis
The differential diagnosis in adults includes lichenification,
most often associated with dermatitis in atopic individuals,
extramammary Paget’s disease, genital warts, vitiligo, non-
pigmented seborrhoeic keratosis and vulvar intraepithelial
neoplasia. Lichen planus can be difficult to differentiate, and
the scarring associated with it may simulate end-stage LS.
Graft-versus-host disease and mucosal pemphigoid have
more in common clinically with lichen planus than VLS,
but are also scarring conditions. Additionally, VLS may
co-exist with lichen planus, producing a confusing clinical
picture [25].
When psoriasis co-exists with VLS, the clinical picture is
also confusing as the skin may not appear white, and the clue
is the textural change, which may be subtle. Similarly, when
it co-exists with vitiligo, textural change and loss of architec-
ture will help to differentiate the two conditions clinically.
Vitiligo lacks the epithelial changes seen in LS, presenting
with sharply marginated white macules that fluoresce under
ultraviolet light. In both cases, if there is doubt, a vulvar
biopsy will confirm the diagnosis.
In the peri- and post-menopausal group, atrophic changes
often co-exist and add some degree of confusion. There may
be reduction in the size of the labia minora and pallor of
the vestibule due to estrogen deficiency, which can simulate
VLS.
The recently described condition vestibular sclerosus is
important to differentiate from VLS. This involves only the
vestibule and does not have the histological features of LS.
There is still some controversy as to whether this condition
is a subset of VLS; however, it is not sensitive to corticos-
teroid treatment [40].
In children, VLS has a characteristic clinical appearance,
and there is little to consider in the differential diagno-
sisother than lichenification and vitiligo. Although licheni-
fied atopic dermatitis can simulate VLSin adults, it is much
less likely to do so in children. Vulvar intraepithelial neo-
plasia, which may have the appearance of a white plaque,
has not been reported in pre-pubertal children. Vitiligo in
children is usually not hard to differentiate because of a lack
of epidermal changes.
It is common to find areas of hyperpigmentation and
benign melanocytic proliferation in LS, and these can some-
times simulate melanoma skin cancer [41]. Where VLS is
associated with malignancy, the histology is often hyperplas-
tic. However, differentiated vulvar intraepithelial neoplasia
may have a subtle appearance and the surrounding LS may
lose its pathognomonic hyaline layer [42].
5 Malignancy After Treatment
Extragenital LS and VLS in adults and children have not
been reported to be associated with squamous malignancy.
Before it was realized that VLS could be adequately treated,
there was a significant association with vulvar malignancy
and many studies quoted a figure of about 5% lifetime risk.
About 60% of vulvar squamous cell carcinomas had his-
tological evidence of adjacent LS [43]. Although a recent
guideline has stated that the risk is small, it is not negligible,
and some authors have called for lifelong follow-up in all
patients [8, 44].
The appearance of a vulvar squamous cell carcinoma can
include nodules, persistent fissures, hyperkeratotic plaques,
non-healing ulcers and fungating tumors. Any change in an
area of LS that does not promptly resolve with potent TCS
treatment mustbe biopsied.
The association of VLS with genital malignancy has very
important implications for management. Patients must be
aware of the risk, be educated about what to look for, and
be regularly treated and followed up.
Anecdotally, experts have long suspected that adequately
treated VLS might have a malignancy rate much lower than
5%. The suggestion has therefore been made that the risk
of malignancy is reduced in uncomplicated VLS that had
been diagnosed and treated appropriately [45]. A prospective
study of VLS in 507 adult women compared patients who
adhered to treatment and those who did not. It demonstrated
that TCS treatment that kept the skin objectively normal also
resulted in minimal scarring and greatly reduced the risk of
cancer [9]. Other authors had previously suspected this, but
it had not been confirmed [45, 46].
6 Management
VLS in adults is a lifelong disease that is unlikely to remit.
Most patients are unable to stop treatment without eventual
relapse, although this may take many months. It is impor-
tant when counseling to emphasize that treatment should be
assumed to be for life [46].
In the unusual instances where patients have apparently
remitted, they need to be kept under long-term observation
Author's personal copy
Vuvlar Lichen Sclerosus: An Update
as, in the authors’ experience, VLS can re-activate after
years of dormancy.
6.1 Severity Grading andTreatment Stratication
Before making a decision about treatment, it is important
to decide on the severity of VLS. In general, the severity
of a skin condition can be determined by a combination of
the inherent observed severity of the disease and its impact
on quality of life. This will give a global impression, but
in terms of treatment selection in VLS, it is the degree of
hyperkeratosis that can be modified by medical treatment
[9]. Following response, there is usually return of function
and reduction in life impact. Although fusionmay partially
reverse, scarring is not affected by medical treatment, and
surgical management is usually required if there is func-
tional impairment. In the author’s experience, not all scar-
ring results in loss of function, particularly if the patient is
not sexually active.
As yet there has not been a study that has resulted in a
universally accepted severity grade. However, two studies
have suggested a grade of hyperkeratosis as a guide to choice
of topical therapy [40, 47].
6.2 Topical Therapy
There are two phases of treatment for VLS:
1. Induction of remission, carried out over a period of up
to a year.
2. Maintenance treatment, which is lifelong.
It is now accepted that potent TCS is the gold standard
for obtaining remission in VLS. The first report of this treat-
ment was published in 1991 (clobetasol propionate 0.05%, a
‘super-potent’ TCS) and many others followed [32, 47, 48].
Until that report [32], it had been considered unthinkable to
apply such strong TCS to genital skin and treatment regi-
mens with weak TCS, testosterone and progesterone were
used. As a result, VLS was considered very difficult to treat.
Once it was shown that potent TCS was effective and safe,
VLS became oneof the easiest vulvar conditions to manage.
Many further studies with potent and super-potent TCS have
confirmed this as a safe and highly effective treatment in
children and adults [31, 44, 49].
VLS is in fact so responsive to TCS that failure to improve
should be reason to suspect that the diagnosis is wrong, the
patient is not using the treatment or there areother factors
confounding symptomatic response, such as malignancy,
allergy, superinfection or estrogen deficiency. In rare cases
of severe hyperkeratotic disease, however, even potent TCS
alone can be inadequate to achieve complete control [50].
Initially, most published data were focused on clobetasol
propionate, with more recent studies comparing its effi-
cacy with that of mometasone furoate 0.1% [48]. However,
it should not be assumed that VLS can only be treated by
these two products, and in almost all cases, other TCS of
similar potency will produce good outcomes [8, 51]. The
clinician should first decide if the lesions are more or less
hyperkeratotic, and match the potency of the topical steroid
to the severity of the skin disease [9]. There is no one prepa-
ration that is superior to the other; the relative severity and
choice of treatment appropriate to it is what is relevant to
management [9]. In general terms, medications in ointment
bases rather than creams tend to be better tolerated and more
effective on the vulva. This has been documented in a recent
study [52].
Therefore, in the authors’ opinion, the main focus of treat-
ment should not be on the product used, but the end result:
attaining and maintaining normal or near to normal skin.
There is no single way to do this, and clinicians can make
their own judgment relative to the severity of the patient’s
disease and their preference for daily or intermittent treat-
ment. Many patients relate that daily regimens are easier to
recall than intermittent ones. Regular follow-up encourages
ongoing compliance.
6.3 Induction ofRemission
Regimes to induce remission vary, but the common theme
is that a potent or super-potent TCS should be used initially.
There is no single way to induce remission, and therapy
should be guided by individual response.
A recent study recommended the following [9]:
Severely hyperkeratotic disease (very thick, white
plaque): ultra-potent TCS (clobetasol propionate 0.05%
ointment) twice daily until itching has ceased (usually
1–2weeks) then daily until review at 6weeks.
Hyperkeratotic disease (moderately thick white plaque):
super-potent TCS (e.g., betamethasone dipropionate
0.05% or mometasone furoate 0.1%) twice daily until
itching has ceased then daily until review at 6weeks.
Mild disease with only pallor and very little hyperkerato-
sis: moderate potency TCS (e.g., triamcinolone acetonide
0.02%, methylprednisolone aceponate 0.1%) daily until
review at 6weeks.
When a potent TCS is used on the vulva, it is important to
review the patient at around 6weeks of treatment to assess
effect and tolerability. Patients may not have understood how
important ongoing treatment is initially, and this visit is an
opportunity to emphasize this. Patients are usually feeling
much better and many assume they are cured. It is important
to emphasize that treatment must now be maintained and
Author's personal copy
A.Lee, G.Fischer
to explain that the reason for this is to prevent cancer and
scarring.
The initial potency of TCS is continued until the skin
texture and color has returned to normal or as close to nor-
mal as possible. There may be residual hyper- or hypopig-
mentation; however, the texture of the surface of the skin
usually improvesmarkedly. Once this has been achieved,
patients progress to maintenance therapy. Symptom resolu-
tion occurs quickly, but resolution of abnormal signs takes
longer. Patients must therefore continue their regular treat-
ment even after symptom resolution.
6.4 Long‑Term Management
Although the correct diagnosis and initial management of
VLS is of great importance, treatment does not stop there,
and indeed it is long-term control that ensures the safety of
the patient [9].
Regimens for maintenance treatment are difficult to
research because of the challenges of conducting a long-term
follow-up study. Typically, reviews and published articles
state that the condition does not spontaneously resolve and
has to be controlled; however, there is no consensus on what
this long-term controlinvolves [5355]. A recent guideline
stated in its introduction that “Treatment remains unsatis-
factory particularly in women as disabling scar formation
is common despite treatment.” The same review stated that
treatment aimed chiefly for suppression of symptoms and
that proactive management may be considered to maintain
remission, if needed, in active disease. No specific recom-
mendation was made [56].
A Cochrane review has stated that there is insufficient
data to make recommendations on long-term management
and called for a study of approximately 2000 women, with
half in an untreated control group to determine whether
treatment could prevent malignancy, but knowing the pos-
sible complications of lack of treatment, this is difficult to
condone ethically [49].
The time taken to achieve remission of VLS is vari-
able, but in the authors’ experience, it is usually around
3–6months of continual potent TCS treatment. The weak-
ness of most published studies is that they observe patients
for the first 3–6months only. Long-term observational stud-
ies of adequate numbers of treated patients are few. For most
publications, the longest period of observation documented
is 3years.
There are three exceptions: a descriptive cohort study
from the UK, with a mean length of follow-up of 66months,
a long-term study from France, which was conducted pro-
spectively over 10years, and a study from Australia con-
ducted prospectively over 8years [9, 45, 46]. This latter
study of 507 women is the best evidence we have to confirm
what most experienced practitioners know: that although
TCS easily induces remission, it does not cure VLS. Fur-
thermore, the French study reported an 84% recurrence rate
if treatment was ceased.
These studies suggest that treatment might change the
course of the disease, reducing the risk of cancer and scar-
ring. The Australian observational study provided compel-
ling evidence that this is the case [9]. In this study, patients
were reviewed every 3–6months and the potency of the
TCS was slowly titrated down to a moderate to mild potency
for maintenance therapy. This was achievable in about 75%
of patients. The rest required long-term therapy with more
potent TCS.
The maintenance regimen used in the French and UK
studies was intermittent clobetasol propionate 1–3 times a
week, and this is what most other published papers have
stated ever since. However, there is no single way to treat
VLS long term, because differing degrees of severity require
different regimes. What is important is to achieve an out-
come of maintenance of normal skin texture and color.
The main problems that have been postulated with long-
term use of TCS on the genital area are TCS-related atro-
phy, peri-orificial dermatitis and Candida superinfection.
In practice, only peri-orificial dermatitis is common and is
frequently asymptomatic as long as the TCS dose is titrated
to severity.
Interestingly, the French and UK studies and another from
the UK with a 3-year follow-up period [51] recorded that
side effects were rare when treating VLS with TCS. This was
confirmed by an Australian study where side effects were
minimal and reversible and were confined to skin fragility
and erythema [9]. The argument that long-term TCS would
produce atrophy is therefore not valid in VLS.
In practice, a treatment review every 6months will deter-
mine the lowest maintenance regimen that will ensure con-
tinuing remission. TCS treatment is constantly titrated to
degree of hyperkeratosis. If this relapses, the strength of
treatment increases. If atrophy or corticosteroid dermatitis
occurs, it is reduced. Managing patients with VLS long term
with TCS has been shown to be safe, inexpensive and effec-
tive [57]. None of the compliant patients in the Australian
study developed a cancer and over 95% had no further dis-
ease progression or scarring. Over 90% had complete and
sustained symptom control, and in those who were sexually
active, over 90% no longer experienced dyspareunia. For the
majority, good compliance was easily achieved.
In the light of this study, regimens that are used on an
“as needed” basis to control symptoms only may need to be
re-evaluated [34]. Symptom control in VLS is not difficult
to achieve, but objective disease suppression should be the
target outcome or the patient is still at risk of complications.
It is a common theme amongst patients who have succumbed
to cancer or disease progression as a result of poor compli-
ance with treatment that they had remained asymptomatic.
Author's personal copy
Vuvlar Lichen Sclerosus: An Update
In children, a recent retrospective study of 46 children
with VLS, comparing compliant patients with non-com-
pliant ones, showed that when normal skin is attained and
maintained, progression of the disease ceased and scarring
and atrophy did not occur. Scarring that was present prior to
treatment, however, did not reverse [36].
A recommended long-term follow-up regimen from the
Australian study is:
Patients are reviewed every 6months until they have been
in a stable remission for 2years, then yearly with the
proviso that they have an examination by their general
practitioner half way through that year and come back
earlier if they have any concerns.
If evidence of relapse occurs on treatment, more potent
corticosteroid is used until this settles.
If there is evidence of corticosteroid excess, less is used.
Corticosteroid excess usually evidences itself with vulvar
redness and burning or fragility. This reverses quickly
once treatment is adjusted.
Patients should be encouraged not to stop treatment once
they are in remission, but to continue with the lowest
dose of corticosteroid possible to maintain complete
objective normality. The psychological impact of a recur-
rence on a patient who is finally in remission after years
of suffering can be devastating. Furthermore, patients
who do not comply with treatment have a 50% risk of
scarring and a 5% risk of development of malignancy.
Each review is an opportunity to remind your patients of
the importance and safety of maintenance treatment.
The main outcome measures of treatment should be:
Symptom control: no itch or soreness is expected.
Ability to have intercourse: in post-menopausal women
this may also require topical estrogen to reduce vaginal
dryness.
Prevention of scarring, fusion and loss of clitoral sub-
stance. (Reduction in labia minora after menopause is
common, not problematic, but not always prevented by
treatment.)
Prevention of malignancy.
Lack of side effects.
6.5 Side Eects ofTreatment
Candidiasis: this is easily controlled with antifungal
therapy.
Erythema: this responds rapidly to a reduction in corti-
costeroidstrength.
Stinging from topical therapy: this usually settles as fis-
sures and erosionsheal. It is virtually always possible to
find a well-toleratedTCS.
Some patients may have recalcitrant thickened areas that
appear non-responsive even to super-potent corticosteroid.
These should always be biopsied to rule out malignancy.
Such lesions may respond to intralesional corticosteroid if
they are causing distress [58].
The most important principle is to maintain follow-up
to maintain treatment and detect complications. It has been
argued by some authors that this approach is unnecessary in
uncomplicated disease [44, 59]. A study from the UK has
shown that once a patient leaves the specialist, VLS is not
well followed up in general practice [60]. VLS is not com-
mon, and the cost of even one patient with a vulvar cancer
or the need for surgery for scarring might be compared to
the cost of follow-up of many to ensure that cancer does not
occur and scarring does not progress.
6.6 Other Topical Therapies
Topical immunosuppressive agents, such as tacrolimus and
pimecrolimus have been described as potentially playing a
role in the treatment of VLS in children and adults [61].
There has been one phase II trial to assess the safety and
efficacy of tacrolimus ointment 0.1% for the treatment of
VLS, and the results were released in 2006. Clearance of
active LS was reached by 43% of patients at 24weeks of
treatment and partial resolution was reached in a further 34%
of patients. Maximal effects of therapy occurred between
weeks 10 and 24 of treatment [62]. The authors who recom-
mend topical immunosuppressives state that these agents
are less likely to cause atrophy. However, atrophy is in fact
rare from TCS, and when it occurs, it invariably improves
with a lower dose.
While there were no adverse events during the 18months
of follow-up, the theoretical disadvantage of topical immu-
nosuppressive agents is an increased risk of malignant trans-
formation due to local immunosuppression. This is arguably
an important consideration given the well-described asso-
ciation of VLS and malignancy. Squamous cell carcinoma
has been reported in adults with VLS in association with
pimecrolimus treatment [63].
At the time of writing, there is insufficient data to recom-
mend topical immunosuppressive agents to treat LS and no
justification when TCS is effective and safe. Topical immu-
nosuppressive agents have no advantage over TCSs. They
are more expensive, very likely to sting and burn, and their
long-term safety is notestablished.
Topical tretinoin has been used in VLS as monotherapy,
but evidence is lacking and treatment is possibly limited by
irritancy [64].
Historically, topical testosterone has been used to treat
VLS, but currently, there is no longer any role for it, with
more effective treatments available and as it may produce
androgenization in girls [65].
Author's personal copy
A.Lee, G.Fischer
Similarly, topical estrogen is of no value, other than to
reduce hypo-estrogenic atrophy in post-menopausal women.
This in fact is an important aspect of managing post-meno-
pausal women with VLS, but in itself is not a specific treat-
ment. However, ongoing pain from vaginal dryness and
fragility which are part of the genitourinary syndrome of
menopause may make it hard to assess outcomes of treat-
ment for concurrent VLS and should therefore be addressed
concurrently.
6.7 Physical Therapy: Surgery, Laser, Phototherapy,
PhotodynamicTherapy, Intralesional
Corticosteroid andLipo‑Injection
Historically, vulvectomy has been performed in adults for
VLS, but the disease recurs despite this. This is no longer
considered an acceptable method of treatment and is com-
pletely contraindicated.
Various surgical procedures have been used to treat labial
and periclitoral adhesions. Simple division of adhesions
gives a very satisfactory result, provided that potent TCS
are used daily post-operatively until healing is complete.
Unless this is done, re-fusion is likely [66]. It is sometimes
necessary to apply the post-operative steroid on a dilator.
Surgery is rarely appropriate therapy in the pediatric popu-
lation unless significant fusion of the labia has occurred.
Newer treatments for very recalcitrant hyperkeratotic
VLS include intralesional platelet-rich plasma [67]. There
have been no published trials. Similarly lipo-injection has
received some attention, but convincing clinical trials are
lacking [68].
In some patients with hyperkeratotic disease, ablative
laser treatment can be a useful adjunct to treatment, but is
not a substitute for topical therapy, which must be continued
subsequently to maintain clinical response [50].
Phototherapy including photodynamic therapy has been
reported to be effective [69, 70], and the authors have found
narrow-band UVB to be effective as an adjunct in recal-
citrant cases. However, phototherapy to the genital area is
difficult and not always practical. There are no clinical trials
to support it. There has, however, been a report of successful
treatment of extragenital LS with narrow-band UVB [71].
A concern with treating a condition with a malignancy risk
with phototherapy is that this may be potentiated.
6.8 Systemic Therapy
It is rare for VLS to be so refractory to treatment that sys-
temic therapy would be required. There is some evidence
that methotrexate combined with pulsed corticosteroid can
be effective in severe generalized LS; however, this has
never been extended to use in VLS [72].
There is no evidence that hydroxychloroquine or systemic
retinoids are useful.
7 The Eect ofLichen Sclerosus onQuality
ofLife andSexual Activity
VLS has a major impact on quality of life [73]. This is
not always improved simply by treating the skin disease.
Returning the skin to normal in VLS does not always ensure
that the impact on the patient has been reduced. Although
pain and itch may have resolved, patients often have ongo-
ing issues: the need for maintenance treatment and regular
examinations can in itself be a burden, particularly for chil-
dren and their families. Concerns about the future, particu-
larly relationships, pregnancy and cancer can affect them
greatly emotionally.
In the authors’ experience, patients with VLS can usually
resume sexual activity with treatment. In postmenopausal
women, this is complicated by changes related to genitouri-
nary syndrome of menopause, and in those with significant
scarring, normal sexual activity may not be possible until
this has been corrected surgically.
In women who have had a long history of painful sex,
there may be significant pelvic floor spasm, and physi-
otherapy to overcome this may be needed. Some develop
an aversion to sexual intercourse and need psychological
help. There are those who decline any help, because their
lack of interest in sex has been legitimized by their disease
[74].
In general, it should be possible to return almost all moti-
vated patients to a normal life as long as a regime can be
found that they find acceptable and easy to comply with.
8 Conclusion
Although the etiology of VLS remains unknown, knowledge
of how to manage it has progressed in the last 5years. Once
thought a difficult to manage condition with a poor progno-
sis, it has emerged that with regular TCS treatment patients
can remain well, returning to a state of normal wellbeing for
long periods of time and that the course of the disease can
indeed be modified by treatment, which is safe and effective
in the long term.
Compliance with Ethical Standards
Funding No sources of funding were used to prepare this manuscript.
Conflict of interest Andrew Lee and Gayle Fischer have no conflicts of
interest that are directly relevant to the content of this study.
Author's personal copy
Vuvlar Lichen Sclerosus: An Update
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... The majority of patients considered that the cause of this disease was the acquisition of infection due to the extramarital sexual act. Relevant reasons for these could be that men tend to have sexual intercourse without protection, with different individuals, increasing the risk of possible bacterial and viral infections, such as Epstein-Barr or Human Papilloma Virus, which can also serve an etiological role in the manifestation of BXO (20). Since other relevant studies corresponding to these findings are not openly available for comparison, further clear and pertinent data are required (20,21). ...
... Relevant reasons for these could be that men tend to have sexual intercourse without protection, with different individuals, increasing the risk of possible bacterial and viral infections, such as Epstein-Barr or Human Papilloma Virus, which can also serve an etiological role in the manifestation of BXO (20). Since other relevant studies corresponding to these findings are not openly available for comparison, further clear and pertinent data are required (20,21). The most frequent comorbidities in the studied group in the present study were diabetes and obesity. ...
Article
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Lichen sclerosus is a chronic disease of unknown etiology that can occur in the genital area of both sexes. Balanitis xerotica obliterans (BXO) occurs only in the genital area of men and typically affects the foreskin, penile glans, and/or the meatus. In the present report, an observational, descriptive, and retrospective epidemiological clinical study of available data between January 2006 and December 2020 in patients diagnosed with BXO from the casuistic of CMI DERMAMED (Târgu Mureș, Romania) was performed. During this 15-year period, 164 patients were diagnosed with BXO, of whom 70 (42.8%) were in the 51-60-year-old age group. They presented at the medical consultation at 3-9 months after onset of symptoms, where 124 (75.6%) the patients were found to be with stage III of the disease. The most important comorbidities were found to be diabetes mellitus and obsesity, who were treated with potent steroids (betamethasone and clobetasol) with favorable results, with only nine (5.48%) cases necessitating surgical treatment. In general, BXO is a rare, acquired, non-infectious and chronically inflammatory (autoimmune) skin disease that has an unknown etiology. Although the risk of developing this condition is particularly high in patients in the 40-60-year-old age group, it can occur at any age. In the present report, the majority of the patients presented after a long period of disease development, such that they were already in advanced stages of the condition with clinically subjective symptoms and severe sexual problems. This delay in diagnosis has several causes, including misdiagnosis, psychological issues, and sexual behavior. Diabetes mellitus and overweight/obesity tended to be important comorbidities of BXO since the majority of the patients were also affected by these conditions. These comorbidities can exert an important pathophysiological influence on BXO. The first-line treatment option for this condition is the local application of potent steroids, with favorable effects. Due to the risk of malignant transformation as a result of BXO, it is recommended that these patients should be followed up for a longer period.
... Chronic genital wounds are present in various dermatological diseases, e.g., lichen sclerosus et atrophicus (LSC). LSC is an autoimmune disease with chronic inflammation of the mucous surfaces of the anogenital area [9] which affects both sexes and can lead to severe scarring and tissue shrinkage when untreated [9]. ...
... Chronic genital wounds are present in various dermatological diseases, e.g., lichen sclerosus et atrophicus (LSC). LSC is an autoimmune disease with chronic inflammation of the mucous surfaces of the anogenital area [9] which affects both sexes and can lead to severe scarring and tissue shrinkage when untreated [9]. ...
Article
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Skin wound repair has been the central focus of clinicians and scientists for almost a century. Insights into acute and chronic wound healing as well as scarring have influenced and ameliorated wound treatment. Our knowledge of normal skin notwithstanding, little is known of acute and chronic wound repair of genital skin. In contrast to extra-genital skin, hypertrophic scarring is uncommon in genital tissue. Chronic wound healing disorders of the genitals are mostly confined to mucosal tissue diseases. This article will provide insights into the differences between extra-genital and genital skin with regard to anatomy, physiology and aberrant wound repair. In light of fundamental differences between genital and normal skin, it is recommended that reconstructive and esthetic surgery should exclusively be performed by specialists with profound expertise in genital wound repair.
... White scales, shiny films, and petechial hemorrhages are important features for the diagnosis of psoriasis. e course of psoriasis vulgaris is slow, and some develop since childhood, lasting for more than ten years or decades, or even protracted for life [4][5][6]. It is prone to recurrent attacks, and there are also a few who are cured without recurrence. ...
... Exclusion criteria: (1) patients who are allergic to the ingredients of traditional Chinese medicine; (2) Women who are lactating; (3) Women who have a fertility plan within one year; (4) Patients with clinical stage of regression; (5) Patients with malignant tumors or mental diseases; (6) Patients with incomplete case data; and (7) Patients in a stress state such as infection and trauma. ...
Article
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This study aimed to investigate the therapeutic effect of ephedra-forsythia-red bean decoction (a formula of traditional Chinese medicine) addition and subtraction treatment of psoriasis vulgaris based on 22 MHz high-frequency ultrasound, so as to provide reference for the selection of traditional Chinese medicine formulas for psoriasis and the clinical application of ultrasound. 80 patients with psoriasis vulgaris with exterior closing and internal depression syndrome diagnosed and treated in the hospital were divided into an observation group (40 cases) and a control group. Patients in the observation group were received ephedra-forsythia-red bean decoction addition and subtraction; and those in the control group were received traditional Chinese medicine of observation group subtraction of raw ephedra, cinnamomum cassia, addition of fineleaf schizonepeta herb, divaricate saposhnikovia root. 22 MHz high-frequency ultrasonography was also performed. The psoriasis area and severity index (PASI) score and efficacy indicators were compared between the two groups. The results showed that the detection rate of nail malnutrition and psoriasis infiltration in psoriasis by high-frequency ultrasound was significantly higher than that by arthroscopy, and the difference was significant (P
... Autoimmune responses govern the histological appearance with epidermal atrophy, hyalinization of the upper dermis and immune cell infiltrate. In patients with chronic disease, sclerotization of the tissue is found [24] . LSC is found in females and males with a ratio of up to 10:1 with increased occurrence in pre-pubertal and post-menopausal women. ...
Article
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Aim: Scarring is a physiological process in adult wound repair. Although keratinocytes and fibroblasts are the main cell types of the skin, they differ in migration behaviour and inflammatory responses depending on their location in the body. The aim of this article is to describe wound repair in genital skin and to depict differences with regard to skin anatomy and cellular responses to inflammatory stimuli in acute and chronic wound healing. Methods: This report reviews data from patients undergoing reconstructive and aesthetic plastic surgery as well as published studies on genital wound repair. Genital surgery comprised plastic reconstructive surgery after urological interventions of biological men and women, tissue from trans-males and trans-females undergoing gender reassignment surgery and tissue from patients undergoing aesthetic genital surgery. The cohort comprised a total of 68 patients (32.9 ± 11.3 years), of which 31 were male (mean 30.4 ± 9.3 years) and 37 were female (34.9 ± 12.5 years; mean ± SD). Results: Wound healing in genital skin markedly differs from other areas of the body due to its anatomical features, microbiome, and elevated hormonal responsiveness. Human genital skin is highly extensible and unusually rich in elastic fibres, and it lacks the mechanical anchorage and tensile properties typical of non-genital regions. Acute injury resolves rapidly due, in part, to rapid resolution of the inflammatory response. In contrast to scarring responses on other body surfaces, genital skin wounding is resolved by shrinkage or fistula formation. Conclusion: The embryological origins of genital skin fibroblasts, together with the gender-specific hormonal environment, contribute to the unique phenotype and healing properties of genital skin. When performing genital surgery, it is of utmost importance to be aware of the differing responsiveness of genital tissue to trauma, surgery, and repair.
... ex., valérate de bétaméthasone à 0,1 %) ou un onguent ultrapuissant 2 fois par semaine. Plusieurs études ont montré l'excellent profil d'innocuité de l'application de corticostéroïdes topiques à long terme sur la muqueuse vulvaire [1][2][3] . Le traitement du lichen scléreux vulvaire est crucial pour maîtriser les symp tômes, prévenir la formation de tissu cicatriciel et réduire le risque de carcinome épidermoïde associé 2 . ...
Article
Introduction: Lichen sclerosus is a chronic inflammatory and atrophic dermatosis affecting preferentially the anogenital region. However, the cutaneous involvement remains less known and studied. Methods: We collected 17 patients to study the clinical and therapeutic features of cutaneous lichen sclerosus. Results: We noticed that the frequency of extragenital involvement in our series is high (about 40%). There is a female predominance (76%), with two infantile cases presenting a severe involvement. On the other hand, the absence of sclerosis, in early forms, does not eliminate the diagnosis. Moreover, breast involvement was frequent (41%) and atypical locations, such as the face, were reported. There was an equal frequency between the diffuse and the localized forms. A genital involvement must imperatively be sought. Conclusions: Our series mention the frequency of isolated cutaneous lichen sclerosus. Clinical presentation can be misleading in the early forms because of lack of sclerosis, variability of localizations, variability of severity, and the absence of anogenital lichen sclerosus.
Article
Background Vulvar diseases are common in the general population and have a negative impact on the quality of life. Objectives To describe our experience as dermatologists in the management of vulvar dermatosis consultations. Methods A retrospective observational study was conducted with patients who attended monographic vulvar consultations over a 5-year period. Clinical information was obtained from the patient’s charts. Results 148 women were studied. Their mean age was 43.24 years (standard deviation: 15.15 years), with ages ranging from 4 months to 80 years. 53.4% of patients took between 2 and 5 years to seek medical attention for the first time. The most frequent diagnosis was lichen sclerosus (41.9%), irritative eczema of the vulva (14.9%), and lichen simplex chronicus (10.1%). 83.8% reported anogenital itching, 66.2% pain, and 45.9% dyspareunia. The most frequently prescribed treatment was ultra-potent topical corticosteroids (clobetasol propionate; 41.2%). Patients with lichen sclerosus were significantly older than those who presented with any of the other diseases. No differences were found in terms of either the time of disease evolution or in symptom presentation. Study limitations Retrospective study. Vulvar diseases with an infectious cause are usually managed in primary care, therefore, were not included. All patients were recruited from a single private hospital which limits the comparisons with the public health system. Conclusions Vulvar diseases frequently occur and are associated with high morbidity. It is essential to promote the development of specific vulvar consultations in hospitals. Specialties such as dermatology, gynecology, urology, or physiotherapy must be part of these units.
Article
Background/objectives: To compare the quality of life in patients with vulval lichen sclerosus (VLS), vulval lichen planus (VLP) and chronic vulvovaginal candidiasis (CVVC), as measured by the Vulval Quality of Life Index (VQLI). Methods: A retrospective, single-centre cohort study was conducted at a combined dermatology and gynaecology practice from March 2018 to November 2021. VQLI scores and patient data were systematically collected and recorded in an online patient database. Treatment regimens were individualised and titrated to clinical response. Results: Over 3 years, a total of 200 women were recruited: 59 with CVVC, 79 with VLP and 62 with VLS. The median duration of follow-up for all patients was 45.43 (16.25-80.89) weeks. At baseline, the median (interquartile range [IQR]) VQLI score was 24.00 (19.00-31.00), 21.00 (12.00-26.00) and 14.00 (7.00-26.00) for CVVC, VLP and VLS, respectively. At follow-up, the median (IQR) VQLI score for CVVC, VLP and VLS was 9.00 (3.00-15.00), 9.00 (3.00-16.00) and 5.00 (2.00-10.00), respectively. All three groups showed a significant improvement in VQLI score (p < 0.0001). At baseline, the highest scoring domains were 'Sexual Function' for CVVC and 'Future Health Concerns' for VLP and VLS. At follow-up, the highest scoring domains were 'Sexual Function' for CVVC and VLP, and 'Future Health Concerns' for VLS. Conclusions and relevance: Vulval disease has an immense impact on QOL, especially in patients with CVVC. The VQLI is useful to clinicians in identifying the unique impact of each vulval condition on a patient's QOL in order to provide better patient-focussed care.
Article
Objectives/purpose: Presenting symptoms of vulvar lichen sclerosus (LS) specific to premenopausal women are not well reported in the literature and may differ from those in postmenopausal women. This study aimed to characterize the presentation of vulvar LS among premenopausal women. Materials and methods: An observational web-based study was conducted in premenopausal women with biopsy-confirmed vulvar LS between the ages of 18-50 years. Participants completed a 28-question survey evaluating characteristics of symptoms, timing of diagnosis, alternate diagnoses, and presence of concomitant autoimmune conditions. Results: Of the 956 responses received, 503 met inclusion criteria of biopsy-confirmed LS and premenopausal status. Average age of symptom onset was 27 years, and average age of diagnosis was 32 years, with a 4-year delay in diagnosis. Symptoms most present were dyspareunia (68%) and tearing with intercourse or vaginal insertion (63%). Symptoms that affect the individual most were also dyspareunia (44%) and tearing with intercourse or vaginal insertion (39%). Symptoms that most frequently prompted patients to seek medical attention were dyspareunia (35%), pruritus (31%) and tearing with intercourse or vaginal insertion (26%). Most common skin changes included hypopigmentation (81%), vulvar fissures (72%), and labial resorption (60%), with fissures affecting the individual the most (48%). Sixty-six percent of the respondents initially received an alternative diagnosis, most commonly vulvovaginal yeast infection (49%). Hypothyroidism was the most common concurrent autoimmune condition (10%). Conclusions: Vulvar LS affects premenopausal women, commonly presenting with dyspareunia and tearing with intercourse. This condition should be considered and evaluated in premenopausal women presenting with vulvar symptoms and sexual pain.
Article
Research objective: to determine the characteristics of nutritional status (NS) in women of reproductive age with typical clinical signs of lichen sclerosus of the vulva (LSV) using key NS parameters. Materials and methods. The study included 75 women with average age 31.3 ± 1.3 years. 50 women were diagnosed with LSV and NS disorders (main group), and 25 women were practically healthy (control group). Information about all patients was collected through direct interviews, clinical examination and study of medical records. The presence of anogenital pruritus, soreness or burning, dryness, dyspareunia, urinary disturbances, perianal and/or intestinal symptoms, inflammatory bowel disease, thyroid dysfunction and tenderness, and other comorbidities were recorded. NS evaluated using anthropometric, laboratory and clinical studies. Body mass index, index of nutritional risk (nutritional deficiency), vitamins, micro- and macroelements value, scatological parameters were determined in all patients, and the intestinal microflora was examined. Results. The results of the study showed a disorder of NS in most patients with LSV by all studied parameters in comparison with the control group. Thus, it was found that 60% of patients with LSV had abnormal body mass index as well as NS was observed in 72% of patients. In addition, patients with LSV also had a higher deficiency of vitamins D and B12, and some important micro- and macronutrients. The results of the coprological study showed the neutral fats in feces, unchanged muscle fibers, extracellular and intracellular starch, decreased levels of bifidobacteria and lactobacilli in 68–74% of patients and opportunistic bacteria and fungi in 30–46% of women in the main group. Conclusions. Thus, the results of this study indicate the association between the abnormal NS and LSV. However, further research is needed to assess the course of the underlying disease and to analyze the metabolic pathways that lead to disorders of lipid, carbohydrate and protein metabolism in target patients, to select of the correct nutrients and alternative treatments.
Article
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Vulval lichen sclerosus (VLS) is a chronic inflammatory skin condition affecting the anogenital area in women. Serious long‐term consequences of VLS include the risk of developing squamous cell carcinoma of the vulva as well as of scarring and alteration of vulval architecture. The treatment of choice for genital lichen sclerosus in females is potent to very potent topical corticosteroids. There are few published data on the course of VLS in pregnancy. We present our experience of managing 33 pregnancies in 29 women with VLS.
Article
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To determine if vestibulovaginal sclerosis and lichen sclerosus (LS) are 2 distinct entities. Biopsies obtained from the vagina or vulvar vestibule that contained abnormal subepithelial collagen were reviewed. Cases were categorized either as LS or vestibulovaginal sclerosis based on presence or absence of basal layer degeneration and lymphocytic infiltrate. Clinical data collected included examination findings, biopsy site and indication, previous vulvovaginal surgery, medications at time of biopsy, vulvar LS, treatment, and response. There were 15 cases with a mean age of 62 yr (range: 32-86 yr); 12 (80%) specimens were from vestibule and 3 from vagina. Nine cases were categorized as LS because of lymphocytic infiltrate in combination with basal layer degeneration, of these 8 had LS elsewhere on vulvar skin. Six cases were classified as vestibulovaginal sclerosis and had an absent or sparse lymphocytic infiltrate and essentially normal epithelium; none of these had vulvar LS. While vestibulovaginal sclerosis and lichen sclerosus are distinguishable clinically and histopathologically, further studies are needed to determine if vestibulovaginal sclerosis is a subset of LS or a different condition.
Article
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Background: There are few published data about the incidence of diagnoses or treatment outcomes, for chronic vulval pain. Aims: To document diagnoses and treatment outcomes in a cohort of chronic vulval pain presentations. Materials and methods: A retrospective case review of the patient database of a private vulval clinic between January 2011 and March 2015. Results: Five hundred and twenty-five out of 3360 patients (15.6%) met the criterion of vulval pain alone. Mean age was 47.1 years (range 17-86). Average duration of symptoms was 60 months (range 3-432). Overall, 277/525 (52.7%) patients had satisfactory responses to appropriate treatment and 90/525 (17%) had partial improvement. A dermatosis was identified in 322/525 (61.3%) patients and of these, 211/322 (65.5%) had satisfactory responses to appropriate dermatological treatment. In the remaining 203/525 (38.7%) the skin was normal. These patients were questioned around the possibility of a neuromuscular cause for their pain, including pre-existing dysfunction, trauma or previous operations involving the spine, hips or lower limbs. There were 181/203 (89%) patients considered to have a neuromuscular cause for their pain and considered suitable for physiotherapy and/or neuromodulating medications. Of these patients, 63/182 (34.6%) had satisfactory responses to this treatment. One hundred and sixty-six out of 525 (31.6%) described vulval pain only during sexual intercourse. There was no statistically significant difference between different diagnoses and responses to treatment between patients reporting dyspareunia only and those sexually active women who did not experience dysparenunia (29/525, 5.5%). Conclusions: The majority of this cohort with chronic vulval pain had a dermatological disease with a smaller proportion caused by neuromuscular dysfunction. Both groups are potentially treatable.
Article
Lichen sclerosus (LS) is a chronic skin disease mainly affecting the anal and genital region. This article is a guideline, which means that the authors looked at all the available evidence about LS in scientific journals, and from this, put together up‐to‐date, evidence‐based recommendations for the management of the disease in adults (18+ years), children (0–12 years) and young people (13–17 years). The guideline was written by a committee of the British Association of Dermatologists, following strict criteria they have for assessing all the different studies on the subject. The guideline helps to guide doctors and nurses working in primary care (which means a patient's first port of call when they have a health problem, e.g. GP clinics, walk‐in centres) as well as in secondary care (meaning where GPs will send people for more specialist care, e.g. hospitals). The guideline covers a range of issues, from treatment options, to gaps in research that need to be addressed. From the guideline, an updated Patient Information Leaflet has been created and is available on the British Association of Dermatologists’ website (www.bad.org.uk). On the basis of these studies and expert consensus, 31 recommendations are made for the diagnosis and management of LS for both general practitioners and secondary care specialists. The recommended patient treatment pathways are presented as flow diagrams for men and women. All the evidence points to a potent topical steroid ointment as the most effective treatment for the condition. This is initially used for 3 months and then the frequency of treatment application is tailored to the individual patient to maintain good control of both symptoms and signs. Nine suggestions for future research studies are made to answer some outstanding questions and to fill gaps in current knowledge. Linked Article: Lewis et al. Br J Dermatol 2018; 178:839–853
Article
Lichen sclerosus (LS) is a chronic inflammatory disease most commonly affecting the genital area of women. LS-associated vulvar neoplasms are known to occur (1). Treatment of LS is thought to possibly reduce malignancy risk. However, whether medical treatment of LS can prevent vulvar neoplasms is unclear (2,3). We performed a single-institution, retrospective chart review to identify vulvar neoplasm occurrence in women with biopsy-proven genital LS and to determine whether a correlation exists between LS treatments and vulvar neoplasm occurrence. This article is protected by copyright. All rights reserved.
Article
The overall objective of the guideline is to provide up‐to‐date, evidence‐based recommendations for the management of lichen sclerosus (LS) in adults (18+ years), children (0‐12 years) and young people (13‐17 years). The document aims to. offer an appraisal of all relevant literature up to July 2017, focusing on any key developments. address important, practical clinical questions relating to the primary guideline objective. provide guideline recommendations and if appropriate research recommendations. The guideline is presented as a detailed review with highlighted recommendations for practical use in primary care and in secondary care clinics, in addition to an updated Patient Information Leaflet (PIL; available on the BAD website, http://www.bad.org.uk/for-the-public/patient-information-leaflets). This article is protected by copyright. All rights reserved.
Article
Background: In patients treated for early-stage squamous cell vulvar carcinoma local recurrence is reported in up to 40% after ten years. Knowledge on prognostic factors related to local recurrences should be helpful to select high risk patients and/or to develop strategies to prevent local recurrences. Objective: This systematic review aims to evaluate the current knowledge on the incidence of local recurrences in vulvar carcinoma related to clinicopathologic and cell biologic variables. Data sources: Relevant studies were identified by an extensive online electronic search in July 2017. Study eligibility criteria: Studies reporting prognostic factors specific for local recurrences of vulvar carcinoma were included. Study appraisal and synthesis methods: Two review authors independently performed data selection, extraction and assessment of study quality. The risk difference was calculated for each prognostic factor when described in two or more studies. Results: Twenty-two studies were included; most of all were retrospective and mainly reported pathologic prognostic factors. Our review indicates an estimated annual local recurrence rate of 4% without plateauing. The prognostic relevance for local recurrence of vulvar carcinoma of all analyzed variables remains equivocal, including pathologic tumor free margin distance <8mm, presence of lichen sclerosus, groin lymph node metastases and a variety of primary tumor characteristics (grade of differentiation, tumor size, tumor focality, depth of invasion, lymphovascular space invasion, tumor localization and presence of human papillomavirus). Conclusions: Current quality of data on prognostic factors for local recurrences in vulvar carcinoma patients does not allow evidence-based clinical decision making. Further research on prognostic factors, applying state of the art methodology is needed to identify high-risk patients and to develop alternative primary and secondary prevention strategies.
Article
Purpose: To assess the effectiveness, tolerability, and convenience of the cream formulation of mometasone furoate 0.1% (MMF) in the treatment of active vulvar lichen sclerosus (VLS) and to compare the cream with the ointment formulation. Methods: The following efficacy parameters were assessed in 27 VLS patients treated with MMF cream for 12 weeks (group A): (i) response rate, (ii) percentage of patients achieving an improvement from baseline of ≥75% in subjective and objective scores, and (iii) mean reduction in subjective and objective scores. These efficacy assessments, as well as those regarding safety and adherence, were compared with the assessments recorded among 37 VLS patients treated with MMF ointment (group B). Results: 59.3% (group A) and 78.4% (group B) of patients were considered responders; 44.4% and 40.7% of patients in group A and 54.1% and 45.9% in group B achieved an improvement of at least 75% in subjective and objective scores, respectively. MMF ointment obtained a significantly higher improvement in symptom scores in comparison with the cream formulation. Conclusions: MMF in ointment formulation seems to be more effective in treating active VLS in comparison with MMF cream. Both formulations are well tolerated and there is no difference in patient adherence and satisfaction.
Article
Vulval conditions may present to a variety of clinicians, such as dermatologists, gynaecologists and general practitioners. Women with these conditions are best managed by a multidisciplinary approach, which includes clear referral pathways between disciplines or access to a specialist multidisciplinary vulval service. Informed consent is a prerequisite for all examinations, investigations and treatments. Consent is particularly important for intimate examinations of the anogenital area, and a chaperone should be offered in all cases. All efforts should be made to maintain a patient's dignity. Depending on symptoms and risk factors, screening for sexually transmitted infections (STI) should be considered. If the patient presents with vulval itch, particularly if also complaining of increased vaginal discharge, vulvaginal candidiasis should be excluded. Sexual dysfunction should be considered in all patients with vulval complaints, either as the cause of the symptoms or secondary to symptoms, and assessed if appropriate. This guideline covers several aspects, such as diagnosis and treatment, of the more common vulval conditions (relatively) often encountered at vulval clinics, i.e. vulval dermatitis (eczema), psoriasis, lichen simplex chronicus, lichen sclerosus, lichen planus, vulvodynia and vulval intraepithelial neoplasia (VIN).