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Table 6.1 (continued)
Presentation Lesion characteristics
Histopathologic
pattern (ISSVD 2006)
Treatment
Psoriasis Chronic pruritus
Usually there are typical
lesion in other anatomical
areas
Bright red, sharply demarcated
lesions
Silver scales are often absent and can
only be apparent in the mons pubis
Acanthotic pattern 1. Low and medium potency topical
steroids
2. Emollients, topical vitamin D
analogues, and calcineurin inhibitors
Atopic and
contact
dermatitis
Acute or chronic pruritus Acute eczema: erythema, oedema,
vesicles/papules
Chronic eczema: excoriations and dry
scaly lichenication of the vulva
Spongiotic pattern Elimination of irritants and/or allergens
Vulvovaginal
atrophy
Dryness, irritation,
dyspareunia, dysuria, and
vaginal discharge
Loss of vaginal rugae, shortened
vaginal length and width
Increases the laxity of the introitus,
reduction in the width of labia
minora, preserving its length
– 1. Avoidance of contact irritants
2. Vaginal moisturizers and low-dose
vaginal oestrogen preparations
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commonly involve the labia majora and are mostly asymmetric (usually more pro-
nounced on the dominant side) (Fig. 6.2). It can also present as hypopigmented
areas that can later maybecome hyperpigmented, as consequence of the inamma-
tory process in long-standing disease. The diagnosis is based on clinical presenta-
tion, leaving the biopsy for exceptional cases. Because LSC can disrupt the normal
skin barrier, there may be superimposed infections, namely, fungal.
The treatment should involve a multitargeted approach with removal of irritating
factors, repair of the skin’s barrier function, reduction of inammation, and disrup-
tion of the itch-scratch cycle [1]. Concerning genital hygiene, avoidance of irritants
and use of barrier protection (e.g. zinc oxide ointment) should be advised.
Considering pharmacological options, moderate to high potency topical corticoste-
roids in an ointment base are the cornerstone of the treatment, such as clobetasol
propionate (applied daily while the symptoms are intense and then for 1 more
month). In severe cases the use of calcineurin inhibitors can be considered. For
disruption of the itch-scratch cycle and to improve sleep quality, a combination of
antihistamines (hydroxyzine), tricyclic antidepressants (amitriptyline), or selective
serotonin reuptake inhibitors (uoxetine, paroxetine) can be used.
As this may be a recurrent disease, maintained treatment may be needed in some
cases. Eviction of triggers is of utmost importance to avoid recurrences.
Fig. 6.1 Lichen simplex
chronicus. Bilateral
involvement of the labia
majora
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6.2.2 Lichen Sclerosus
Lichen sclerosus (LS) is a chronic inammatory disease that affects the genital
and perianal areas. Extragenital lesions can be found in about 11–20% of patients
(mainly in the chest, upper back, arms, neck, and buttocks) [9]. The true preva-
lence of LS remains unknown, but recent data showed that it is more common
than previously described, with a prevalence of 1:60 [10], and when considering
older populations, it can be as high as 1:30 [11]. It can be seen at any age, but
usually the distribution of prevalence has two peaks: one prepubertal (7–15% of
all cases) and the other at the fth and sixth decades of life, comprising the major-
ity of cases [12].
While there are several risk factors andconditions known to be linked to LS, its
aetiology is still not completely understood [1]. Its association with autoimmunity is
well recognized, particularly with thyroid disease (until 30%), but also other disorders,
Fig. 6.2 Lichen simplex
chronicus, predominantly
on the left side
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such as alopecia areata, pernicious anaemia, morphea, vitiligo, diabetes mellitus,
inammatory bowel disease, celiac disease, and psoriasis. Autoantibodies are fre-
quently positive—extracellular matrix 1 protein, anti-basement membrane, and anti-
thyroid antibodies. There is also a genetic association, with a positive familial history
in 17% of cases. The hormonal milieu has also been implicated, with hypoestrogenism
and a diminished number of androgenic receptors consideredto be possiblecontribut-
ing factors—however, lack of response to hormonal treatments (such as oral contracep-
tives and menopausal hormone therapy) gives no consistency to this theory [13]. The
presence of traumatic lesions induced by burning, friction, and scars is a frequent target
to the appearance of LS lesions—Köbner or isomorphic phenomenon [12].
The most common symptomatology is vulvar pruritus and/or burning sensation
in the vulvar and perianal area, worsening at night time. Some women also refer
pain, soreness, dyspareunia, and dysuria. They may present scratching habits, but
they do not refer relief with it, contrary to what happens in LSC.They can, however,
develop LSC secondary to the repetitive scratching. It is important to recognize that
at least15–40% of the women areasymptomatic [10, 14].
Physical examination may reveal reddish papules or ivory patches, with hyper-
keratosis that can coalesce and form plaques, usually symmetrical. It is also com-
mon to nd ssures, petechiae, purpura, erosions, ulcerations, oedema, hypo- and
hyperpigmentation, and “cigarette paper wrinkling” aspect of the skin (Fig.6.3).
Fig. 6.3 Lichen sclerosus.
Atrophy of the labia
minora, phimosis, cigarette
paper wrinkling, and
hypopigmentation
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The lesions are typically conned to hairless vulvar areas (inner portion of labia
majora, labia minora, vestibule, clitoral hood) and frequently (30%) perianal area.
When there is simultaneous involvement of vulva and perianal area, a “gure of 8”
or “key hole sign” is the characteristic observed feature (Fig.6.4). In cases of long-
standing disease, radical anatomical changes can occur, such as introital stenosis,
phimosis, fusion (Fig.6.5), and resorption of the labia minora, with both a reduction
in width and length (in senile atrophy of the labia minora, usually the length is pre-
served), and all these alterations can be asymmetrical. Vaginal involvement is
exceptional and seems to be associated with vaginal walls prolapse (Fig.6.6) [15].
Sexual dysfunction is common among women with LS.Dyspareunia and symp-
toms derived from distortion of the vulvar anatomy are frequently referred. The
phimosis and consequent burying of the clitoris can lead to the formation of smeg-
matic pseudocysts that can become enlarged, painful, and infected. Also, the forma-
tion of synechiae over the urethral meatus or the fusion of labia minora can cause
urinary stream alterations and recurrent urinary tract infections (Fig.6.5).
The most fearful complication of LS is vulvar intraepithelial neoplasia (VIN)
and vulvar cancer. Although LS is not considered a premalignant condition, it has
Fig. 6.4 Lichen sclerosus.
“Figure of 8” or “key hole
sign”
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been associated with an increased risk of keratinized vulvar squamous cellcarci-
noma (2.6–6.7%) [12, 16]. The truly premalignant lesion associated with LS is VIN,
differentiated type (dVIN). It is much rarer (2–10%) [17] than the HPV-associated
vulvar high-grade squamous intraepithelial neoplasia (HSIL), but it is responsible
for the majority of vulvar cancers. Those are indolent lesions, with a 10-year risk of
progression estimated in 18% [12].
The diagnosis of LS is clinical. While some authors recommend routine biopsy
in all cases, most disagree and recommend it only in cases of treatment failure,
when second-line therapy is needed, or to exclude dVIN or cancer. Suspicious
lesions that should prompt a biopsy are erosions/ulcers, hyperkeratosis, or pig-
mented areas. It is important to note that biopsy does not guarantee a nal diagnosis
of LS, especially in patients already under steroid treatment. Of note, the same area
or lesion may have to be biopsied along the time, if suspicion persists [12].
Fig. 6.5 Lichen sclerosus.
Almost complete fusion of
the labia minora
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As part of the autoimmune diseases workout, we recommend routine testing of
thyroid function, including T4, TSH, and thyroid antibodies [12].
The treatment aims to control the symptoms, prevent anatomical distortion, and
diminish the risk of malignancy. In about one-fth of the cases, total remission can
be observed [18]. Women compliant to the prescribed treatments are less likely to
develop vulvar cancer, reinforcing the importance of treating even asymptomatic
women and leading to the discussion on the need of maintenance therapy in asymp-
tomatic women [1, 18, 19].
1. General care: it is essential to break the itch-scratch cycle to minimize all pos-
sible trauma to the genital area. As so, it is important to advise against multiple
washing and use of irritating products (soaps) and to avoid factors that can
worsen the symptoms (urinary incontinence, menstruation).
2. Topical treatments: ultra-potent topical steroids are the mainstay of the treatment
for LS.Clobetasol propionate (CP) ointment 0.05% is the most used one, and its
efcacy is due to anti-inammatory, vasoconstrictor, and antipruritic proprieties.
The authors advocate a scheme of application of CP once a day for 4weeks, fol-
lowed by once every other day for another 4weeks, and nally twice a week for
another 4weeks, completing 3months of initial treatment. Afterwards, it should
Fig. 6.6 Lichen sclerosus.
Unusual case of LS with
involvement of the anterior
vaginal wall
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be used the lowest dose possible for symptomatic control (application 2–3 times
a week), with a maximum dosage of 60–120g/year. Everyday schemes for more
than 3–4weeks are not advised, not only because of the risk of complications but
also due to that of tachyphylaxis [20]. As second-line topical treatment—when
topical steroids are not well tolerated or there is no response—calcineurin inhibi-
tors can be considered, starting by applying one to two times daily and then
reducing the frequency for long-term maintenance. Those are immunomodula-
tors agents that block the release of inammatory cytokines from T lymphocytes
and are efcient in controlling the signs and symptoms of the disease. Moreover,
CP is faster in controlling the symptoms and is associated with higher likelihood
of achieving complete remission [21].
3. Systemic treatments: reserved for severe LS, nonresponsive to topical treat-
ments. Options include a combination of steroids plus methotrexate, retinoids
(acitretin, isotretinoin, or etretinate), methotrexate alone, and cyclosporine. In
any case, before initiating one of these options, it is mandatory to have a histo-
logical diagnosis. Oral tricyclic antidepressants (amitriptyline, 10 mg), anti-
histaminic drugs, or local anaesthetics may be used to control the symptoms.
4. Surgical treatment: it should be reserved for the treatment of complications, such
as urinary obstruction, introital stenosis, synechiae causing symptoms, and
symptomatic smegmatic pseudocysts, or treatment of premalignant or malignant
lesions. It is important to note that disease tends to relapse in scars andeven in
grafted tissue [12].
A close follow-up is necessary, with the rst visit after beginning the treat-
ment no later than 3months and the following one no longer than 6months. The
goal of treatment should be controlling the symptoms and normalization of skin
colour and texture [22]. There is a risk of vulvar cancer throughout the woman’s
life, which signies that the follow-up must be kept indenitely, independently
of being symptomatic or not—at least every 6–12months.
Recently, there have been some papers suggesting the use of adipose-derived
stem cells and platelet-rich plasma and even LASER for the treatment of LS.Up
to now, the available evidence does not sustain those claims, and development of
LS has even been associated with Nd:YAG LASER therapy for hair removal [
23,
24]. In our opinion, and in line with the ISSVD and International Continence
Society position, these treatments should be avoided and only used in the context
of clinical trials [25].
6.2.3 Lichen Planus
Lichen planus (LP) is an inammatory disorder that affects mucocutaneous tissues.
Vaginal involvement is common, as well as that of other non-genital regions, includ-
ing the oral mucosa (Fig. 6.7), skin, nails, oesophagus, and conjunctiva. Among
women with oral disease, up to 75% have genital involvement [26]. Vulvovaginal
LP can be divided in three forms: erosive LP, papulosquamous LP, and hypertrophic
LP.Erosive LP is the most common variant that affects the vulva. The prevalence of
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LP is estimated to be of 0.5–3.7% [27], and it mostly affects women between 30 and
60years old.
LP is considered an autoimmune disease with a T-cell-mediated pathogenesis,
and the interaction with some exogenous factors (nonsteroidal anti-inammatory
and antihypertensive drugs and, in oral LP, hepatitis C virus [28]) may also play a
role. However, the exact aetiology remains unclear, and despite the associations
described, recommendations for screening are lacking in asymptomatic women [1].
The symptoms are similar to those described for LS, namely, dyspareunia and
burning. Vaginal discharge and postcoital bleeding are also common complains.
On physical examination, the presence of white, reticulate, lacy, or fernlike striae
(Wickham striae) adjacent to erythematous epithelium is typical, and they often
affect the medial aspect of the labia minora and the vulvar vestibule (Figs.6.8 and
6.9) [29]. Also, the presence of pruritic, purple, shiny papules may be seen, but
they are dusky pink in colour, without an apparent scale and less well demarcated
than on other areas of the body. Contrary to LS, vaginal involvement is frequent,
and the epithelium can have an erythematous, eroded, and acutely inamed aspect.
The use of wet mount microscopy can be useful to evaluate vaginal involvement
and monitor the response to treatment (Fig.6.10). In the posterior vestibule and
labia minora, deep, painful, erythematous erosions may be apparent, and they
result in a resorption of labial architecture (Fig. 6.9). These erosive lesions are
extremely friable, and, over the time, the surfaces may adhere resulting in syn-
echiae and, eventually, complete obliteration of the vagina [3]. The examination of
the oral cavity is mandatory, and in woman with genital LP, up to 70% have oral
disease (Fig.6.7) [30]. There are some cases of asymptomatic disease, but it seems
to be rare [31]. The diagnosis is clinical, and biopsy is considered for dubious cases
in which it is necessary to rule out neoplastic conditions, in which there is no
response to treatment, or when a diagnosis of some autoimmune blistering diseases
is also being considered [1].
Fig. 6.7 Lichen planus.
Oral involvement in a
woman who also has
vulvar LP
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General care management measures are similar to those previously described for
LS.If these patients are taking nonsteroidal anti-inammatory or antihypertensive
drugs, if possible, it should be stopped temporarily, to check if there is improve-
ment. The rst-line treatment is, as in LS, the ultra-potent topical steroids, and the
application scheme could be the same proposed for the treatment of LS.Second-
line options include calcineurin inhibitors, systemic steroids, immunomodulators,
methotrexate, and retinoids. The use of vaginal dilators may be considered to pre-
vent vaginal adhesions derivedfrom the coaptation of theinamed vaginal mucosa.
It can be coated with a corticosteroid ointment and oestrogen vaginal cream. Usage
can be daily or every other day, according to the response [32]. Surgery is reserved
to restore the vaginal vault.
The follow-up schedule is similar to that described for LS.In patients with LP,
the risk of cancer of the lip, tongue, oral cavity, oesophagus, and larynx is increased
[33], but the occurrence of neoplastic transformation for vulvar LP is controversial.
The standardized incidence ratio of vulvar cancer in women has been estimated to
double in women with LP [33], and these cancers seem to be more aggressive and
more likely to recur [34]. A recent study, however, did not observe any case of HPV-
independent vulvar squamous cell carcinoma in women with LP [35]. Of note, Preti
et al. have associated higher risk of recurrence of vulvar HSIL in women with
Fig. 6.8 Lichen planus.
Erythema of the vestibule
and partial resorption of
the labia minora
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