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430 Non-neoplastic Lesions of the Vulva
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Non-neoplastic Lesions of the Vulva (Inflammations,
Dermatologic Conditions, Infections), Pathology
of the Vulva, Fig. 10 The physical exam findings vary
somewhat depending on chronicity. In acute eczematous
dermatitis, vulvar erythema, edema, and vesicles can be
seen. (Courtesy of Dr. Libby Edwards)
hyperpigmentation or hypopigmentation, may
develop (Pichardo-Geisinger 2017).
• Incidence
Incidence of vulvar eczematous dermatitisin the
general population has not been formally studied. Vulvar contact dermatitis has a reported
incidence in vulvar clinics of 20–30% in the
United Kingdom and 15% in Australia (Crone
et al. 2000; Brenan et al. 1996).
• Age
Women of all ages can be affected by vulvar
eczematous dermatitis; however, etiology
varies with age. Children are more likely to
present with atopic and irritant dermatitis,
while allergic contact dermatitis is very rare
in this age group (Fischers and Rogers 2000).
In adults, both allergic and irritant contact dermatitis are common.
• Site
In contact dermatitis, the affected sites are typically localized to the area of exposure. Extension to the perianal area and inner thighs can
also be seen.
• Treatment
An important factor in the management of
eczematous dermatitis is eliminating irritant
and allergen exposure, which may include
Non-neoplastic Lesions of the Vulva (Inflammations,
Dermatologic Conditions, Infections), Pathology
of the Vulva, Fig. 11 Subacute to chronic stages are
characterized by erythematous to lichenified and excoriated patches and plaques often with scaling and accentuated skin markings. (Courtesy of Dr. Libby Edwards)
overwashing, lubricants, topical hygiene prod-
ucts, urine, feces, bubble baths, and vigorous
scrubbing with loofahs and towels. Identifica-
tion of the precise offending agent is challeng-
ing; patch testing can be considered in allergic
contact dermatitis. Topical anti-inflammatory
agents including topical corticosteroids and
topical calcineurin inhibitors are commonly
used. Controlling pruritus with antihistamines
can aid in breaking the itch-scratch cycle. In
severe cases of contact dermatitis, a short
course of systemic steroids may be necessary
(Pichardo-Geisinger 2017).
Macroscopy
Appearance depends on severity of exposure,
location, and evolution. The acute stage present
with well-demarcated erythematous and/or edematous plaques with erosions. The intraepidermal
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Non-neoplastic Lesions of the Vulva (Inflammations,
Dermatologic Conditions, Infections), Pathology
of the Vulva, Fig. 12 The histologic findings of eczem-
atous dermatitis are similar regardless of etiology.
Spongiosis, or intraepidermal edema, characterized this
inflammatory pattern
edema may collect resulting in the formation of
vesicles and bullae. The erythema decreases in
subacute stage and small dry scales may appear.
The chronic stage is characterized by lichenified
plaques with scale.
Microscopy
The histologic findings of eczematous dermatitis
are similar regardless of etiology. The lesions are
characterized by spongiosis, or intraepidermal
edema (Fig. 12), with a perivascular dermal infiltrate composed of lymphocytes and occasionally
eosinophils. Histologically, spongiotic dermatitis
can be classified as acute, subacute, or chronic
phase. In acute spongiotic dermatitis, spongiosis
is most pronounced and may develop
intraepidermal spongiotic vesicles with
Langerhans cells (Fig. 13). Langerhans cell collec-
tions in the epidermis also known as Langerhans
cell granulomas and dyskeratotic cells may be
seen. Subacute spongiotic dermatitis may show
serum crust and mild epidermal acanthosis.
Chronic spongiotic dermatitis displays superimposed changes of lichen simplex chronicus
including acanthosis, hypergranulosis, and superficial dermal fibrosis. Spongiosis may be minimal or
absent in the chronic phase (Hoang et al. 2014;
Taylor 1986;ChanandZimarowski2015).
Non-neoplastic Lesions of the Vulva (Inflammations,
Dermatologic Conditions, Infections), Pathology
of the Vulva, Fig. 13 In acute spongiotic dermatitis,
spongiosis is most pronounced and may develop
intraepidermal spongiotic vesicles
N
Non-neoplastic Lesions of the Vulva (Inflammations,
Dermatologic Conditions, Infections), Pathology
of the Vulva, Fig. 14 The presence of eosinophils
among the inflammatory dermal infiltrate may suggest an
allergic etiology
The presence of Langerhans cell granulomas
and eosinophils among the inflammatory dermal
infiltratemay suggest an allergic etiology (Fig. 14).
Intraepithelial neutrophils and/or the presence of
necrotic keratinocytes are more suggestive of irritant contact dermatitis (Taylor 1986). These are
some important histopathologic clues that can suggest a particular etiology; however, it is important
to remember that histopathology alone is insufficient to identify the underlying cause. Careful clinicopathologic correlation is necessary.
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Differential Diagnosis
Fungal infection, including Candida and dermato-
phyte, is an important diagnostic consideration and
mayevenbeseensuperimposedoneczematous
dermatitis. Periodic acid-Schiff (PAS) stain can
aid in excluding fungal infection. Acute
vesiculobullous forms of eczematous dermatitis
may mimic other bullous disorders, which can be
excluded with direct immunofluorescence (DIF).
Herpes Simplex Virus Infection
Synonyms
Anogenital herpesviral infection; Genital herpes;
Herpes genitalis.
Definition
Skin lesions produced by infection with herpes
simplex virus (HSV) types 1 or 2.
Clinical Features
• Incidence
HSV represents the most common cause of
genital ulcerative disease in developed countries with a seroprevalence in the USA of
20–50% and 16% for HSV2 and HSV1,
respectively, and about 750,000 new cases/
year (Brown et al. 1999). While HSV2 has
been in the past the most common cause of
genital herpes, an increasing inci dence of
HSV1 genital infections have been observed
recently (Bernstein et al. 2013).
• Age
HSV1 occurs mostly in patients <10 years old
while HSV2 tends to infect young adults and
teenagers often coinciding with the onset of
sexual activity.
• Sex
HSV1 affects equally both genders while
HSV2 is slightly more common in women.
• Site
HSV infection is common on the genital skin
and oro-labial region.
• Treatment
Treatment is with topical, oral, or intravenous
antiviral agents including acyclovir,
valacyclovir, or famciclovir.
• Outcome
HSV replicates at the site of inoculation and
then travels via axons to the dorsal root
ganglia where is stays dormant. From there,
it can reactivate in about 90% of cases, usually
during time of stress such as exposure to UV,
fever, or immunosuppression states. About a
third of patients s uffer from multiple recur-
rences. Recurrences are more common for
HSV2 infection compared to HSV1. Compli-
cations including urethritis, proctitis, cystitis,
andmeningitiscanalsooccur(Coreyetal.
1983). Neonatal infection can lead to aseptic
meningitis, and encephalitis, and could result
in infant death.
Macroscopy
HSV infection presents as crops of vesicles on an
erythematous base. In the case of primary infection, it debuts usually 2–14 days after exposure,
sometimes accompanied by prodrome symptoms
including fever, myalgia, malaise, headache, and
back pain (Bernstein et al. 2013; Brown et al.
1999). The lesions evolve to pustules, erosions,
or ulcerations. Exophytic variant of HSV can be
encountered in patients with immunosuppression
or HIV infection and present with verrucous-like
lesions (Garib et al. 2013).
Microscopy
Intact blisters show an intraepi dermal vesicle
accompanied by ballooning and reticular degeneration of the epidermis with acantholysis
(Fig. 15). HSV cytopathic inclusions are noted
in the nuclei of keratinocytes consisting in margination of nuclear chromatin with ground-glass
intranuclear inclusions, multinucleation, and
nuclear molding (Fig. 16). An inflammatory
component with neutrophils may also be
present.
Immunophenotype
Immunohistochemical stains for HSV1 and HSV2
are available for diagnosis and are helpful in cases
without obvious viral inclusions (Fig. 17).
Molecular Features
HSV is a double -stranded enveloped DNA virus.
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Non-neoplastic Lesions of the Vulva (Inflammations,
Dermatologic Conditions, Infections), Pathology
of the Vulva, Fig. 15 Low-power view of an intact
HSV lesion showing an intraepidermal vesicle with reticular degeneration and necrosis
Non-neoplastic Lesions of the Vulva (Inflammations,
Dermatologic Conditions, Infections), Pathology
of the Vulva, Fig. 16 At higher magnification, there are
keratinocytes showing margination of nuclear chromatin,
ground-glass nuclear inclusions, multinucleation, and
molding
Non-neoplastic Lesions of the Vulva (Inflammations,
Dermatologic Conditions, Infections), Pathology
of the Vulva, Fig. 17 Immunohistochemical stain for
HSV2 showing positivity in the nuclei of infected
keratinocytes
N
Non-neoplastic Lesions of the Vulva (Inflammations,
Dermatologic Conditions, Infections), Pathology
of the Vulva, Fig. 18 Exophytic HSV showing epider-
mal hyperplasia with irregular elongation of rete ridges and
areas of epidermal ulceration with underlying dermal
necrosis and inflammation
Differential Diagnosis
The exophytic variant of HSV can be misdiagnosed as squamous cell carcinoma (Figs. 18
and 19) (Lautenschlager et al. 2008; Strehl et al.
2012). In some cases, HSV infection is accompa-
nied by a CD30-positive lymphocytic infiltrate
and mimics lymphomatoid papulosis. Finally,
autoimmune blistering diseases such as pemphigus vulgaris and pemphigus vegetans enter the
differential diagnosis.
Hidradenitis Suppurativa
Synonyms
Acne inversa; Verneui l’s disease.
Definition
Hidradenitis suppurative is a chronic relapsing
skin disease localized to intertriginous areas with
characteristic clinical findings . Hidradenitis
suppurativa is a component of the follicular
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Non-neoplastic Lesions of the Vulva (Inflammations,
Dermatologic Conditions, Infections), Pathology
of the Vulva, Fig. 19 Exophytic HSV showing at higher
magnification multinucleated keratinocytes with multinucleation, molding, and ground-glass appearance, consistent with herpetic viral cytopathic effect
occlusion tetrad, which also includes acne
conglobata, dissecting cellulitis of the scalp, and
pilonidal cyst (Alikhan et al. 2009).
Non-neoplastic Lesions of the Vulva (Inflammations,
Clinical Features
Hidradenitis suppurative diagnosis is made clinically; however, delay in accurate diagnosis is
common (Stewart 2017;Saunteetal.2015).
Dermatologic Conditions, Infections), Pathology
of the Vulva, Fig. 20 Chronic draining nodules and
scarring localized to areas of apocrine glands are typical
of hidradenitis suppurativa. (Courtesy of Dr. Libby
Edwards)
Diagnosis involves the presence of typical
lesions (such as nodules, sinus tracts, or scars)
that are chronic or recurrent and involve the
typical anatomic regions (Zouboulis et al. 2015;
Fig. 20).
The disease exists on a spectrum with clinical
findings varying widely in severity. Several staging systems have been proposed to assist in
assessment of severity and selecting appropriate
therapies. The Hurley staging system is most
widely used at this time. The Hurley staging system classifies patients into one of three groups
based on clinical severity. Hurley stage
I includes patients with transient, nonscarring
inflammatory lesions. Hurley stage II includes
recurrent abscesses with tunnel formation and
scarring. Single or multiple lesions may be present; howe ver, multiple lesions should be separated by normal-looking skin. Hurley stage III
includes patients with coalescent lesions with tunnel formation and scarring (Hurley 1989).
The pathogenesis was initially thought to be a
primary inflammatory disorder of apocrine
glands; however, several subsequent studies
identified apocrine gl and inflammation only in
the presence of extensive inflammation
supporting the idea that this represents secondary
involvement (Anderson Jr and Dockerty 1958;
Yu a nd Co ok 1990). Follicular occlusion with
subsequent rupture of follicles and inflammatory
response is now considered to play critical role in
the development of hidradenitis suppurativa
(Yu and Cook 1990; Jemec et al. 1996). Ult imately, the pathogenesis of hidradenitis
suppurativa is not fully understood, but it is
likely multifactorial with dysfunction of the
folliculosebaceous unit, hormones, genetics,
and immune dysregulation playing a role. Other
important risk factors for the development of
hidradenitis suppurativa include obesity and
smoking (Alikhan et al. 2009).
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Clinical Features
• Prevalence
The overall reported prevalence of hidradenitis
suppurativa is 1–4% (Alikhan et al. 2009;
Jemec et al. 1996; Revuz 2009). The prevalence decreases with age, and individuals
55 years of age or older showed a significantly
lower prevalence than younger indiv iduals
(0.5% vs 1.4%) (Revuz et al. 2008).
• Age
Postpubertal males and females are affected
with onset typically in the second to third
decade of life (Alikhan et al. 2009; Garg et al.
2017). Onset after menopause is rare.
• Sex
Regarding hidradenitis suppurativa in general,
females are more commonly affected than
males (3:1) (Stewart 2017).
• Site
Hidradenitis suppurativa involves
intertriginous, apocrine gland-bearing areas
including axilla, perianal, perineum/groin,
and inframammary regions. In one study,
involvement of the perineum and groin was
reported in 24% of patients with hidradenitis
suppurativa (Jackman and McQuarrie 1949).
• Treatment
There is no uniformly effective therapy for
hidradenitis suppurativa, and treatment
requires a multifactorial approach with modalities chosen based on patient symptoms and
severity. General recommendations for all
patients with hidradenitis suppurativa include
weight loss, avoidance of tight clothing,
smoking cessation, reassurance, and nonnarcotic analgesics. Topical antibiotics are
often recommended in mild disease. Other
therapies that have been used include hormonal therapy, retinoids, immunosuppressive
therapy, and CO
laser ablation (Alikhan et al.
2
2009). Advanced stage disease often requires
wide surgical excision. In the vulva, limited or
local excision has been associated with recurrence of disease (Wood 2015).
• Outcome
This is a chronic disorder that can cause scarring, fistulas, and dyspigmentation. The excessive scarring and fibrosis can cause
contractures, and involvement of the groin/per-
ineum can lead to anal or urethral strictures
(Alikhan et al. 2009). Acute or chronic vulvar
edema has also been described (Stewart 2017).
Hidradenitis suppurativa has a significant
impact on patients’ quality of life and self-
esteem. Involvement of the groin/perineum is
associated with sexual distress and dysfunction
(Matusiak et al. 2010; Esmann and Jemec
2011; Kurek et al. 2012).
Squamous cell carcinoma has been reported
as a rare complication of long-standing
hidradenitis suppurativa. This complication
has most frequently been described in men in
the perianal region; however, a recent review
of squamous cell carcinoma complicating
hidradenitis suppurativa found external female
genitalia to be the site of involvement in 5% of
cases reported in the literature (Jourbachi et al.
2016; Revuz 2009; Manolitsas et al. 1999;
Williams et al. 1991).
Macroscopy
As previously described, physical exam findings
of hidradenitis suppurativa depend on severity of
disease. Clinical exam findings that are common
in hidradenitis suppurativa include comedones,
acneiform pustules, deep-seated nodules or
abscesses, sinus tracts with or without serous or
purulent drainage, erosions or ulcers, and scarring. Scarring may present as hypertrophic or
keloidal. Double or multiheaded comedones are
a characteristic lesion of hidradenitis suppurativa.
Chronic disease may also lead to dyspigmentation
and lichenification. Complications include strictures, contractures, or fistulas (Stewart 2017).
Microscopy
Hidradenitis suppurativa of the vulva is histologically identical to other sites of involvement. Histologic findings include cysts or sinus tracts in the
dermis lined by squamous epithelium and
containing laminated keratin (Fig. 21a, b). These
may contain hair shafts (Yu and Cook 1990).
Disruption of cysts or sinuses may occur and
leads to the development of suppurative and histiocytic inflammation with foreign body giant cell
reaction (Fig. 22). The dermis and subcutaneous
N
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Non-neoplastic Lesions of the Vulva (Inflammations,
Dermatologic Conditions, Infections), Pathology
of the Vulva, Fig. 21 Histologically, hidradenitis
Non-neoplastic Lesions of the Vulva (Inflammations,
Dermatologic Conditions, Infections), Pathology
of the Vulva, Fig. 22 Disruption of cysts or sinuses
may occur and leads to the development of suppurative
and histiocytic inflammation with foreign body giant cell
reaction
suppurativa of the vulva includes cysts or sinus tracts in
the dermis (a) lined by squamous epithelium and
containing laminated keratin (b)
syphilis. Correlation with culture, resolution
with appropriate antibiotics, and careful clinical
correlation with characteristic lesions, age of
onset, and clinical course can aid in rendering
the correct diagnosis. Special stains can be considered to help exclude infectious causes. Crohn
disease may also enter the differential diagnosis.
Evaluation for gastrointestinal disease should be
considered; however, vulvar involvement of
Crohn disease may precede gastrointestinal symptoms. Involvement of other sites characteristic of
hidradenitis suppurativa can also be helpful
(Stewart 2017).
Lichen Sclerosus
Synonyms
Lichen sclerosus et atrophicus.
tissue demonstrate fibrosis and “mixed inflammatory infiltrate” as opposed to infiltration with possible abscess formation. Inflammation of both
apocrine and eccrine sweat glands may be seen;
however, this is now considered to be a secondary
phenomenon (Jemec et al. 1996).
Differential Diagnosis
Various infections may enter the differential diagnosis including abscess, furuncle, granuloma
inguinale, lymphogranuloma venereum, and
Definition
Lichen sclerosus (LS) is a T-cell mediated chronic
inflammatory dermatitis with a predilection for
the vulva. Extragenital involvement is present in
11–13% of reported cases (Cooper et al. 2004;
Simpkin and Oakley 2007; Thomas et al. 1996).
The postulated physiopathology of LS is an
epitopic alteration of the basal layer of the epithelium that triggers a cyclic lymphocytic attack of
damage and repair. The physiopathology behind
this process is poorly understood. It has been
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suggested as a multifactorial disease occurring in
a genetic susceptible patient with elicit factors like
vulva environment, inflammation, and infection
(Fistarol and Itin 2013). LS is an autoimmune
disease with preference for a Th1 immune
response against ECM1 (extracellular matrix protein 1, an essential scaffolding glycoprotein key to
dermal equilibrium). Additional immune and
genetic targets including miR-155, downstream
targets of ECM1, galectin-7, p53, and epigenetic
modifications to be involved as etiologic or accelerating agents (Tran et al. 2019). Other triggers
include trauma or Koebner phenomenon (e.g.,
development of a lesion in normal skin after
scratching or other trauma, as LS can be associated with scars, site of injections, radiotherapy,
and tattoos) (Tegner and Vrana 2001; Yates et al.
1985; Monteagudo et al. 2010; Arun et al. 2010).
An autoimmune etiology has alsobeen considered
as patients suffer also from concomitant autoimmune diseases (e.g., vitiligo, thyroid disorders,
type I diabetes, alopecia areata, lupus
erythematosus, and pernicious anemia) (Meyrick
Thomas et al. 1988; Powell et al. 2000), and they
have tissue-specific antibodies (Goolamali et al.
1974). Moreover, the antigen-mediated immune
response resulting in vasculitis is reinforced by
the presence of periv ascular antigen presenting
follicular dendritic cells interacting with antigenspecific T and B cell lymphocytes (Regauer et al.
2004). Endothelial dysfunction may also play an
important role in tissue injury and fibrotic
response. LS is also defined as an “inflammatory
scar” (Carlson et al. 1998).
intermittent or constant (Thorstensen and
Birenbaum 2012 ). Women may also experience
a burning pain due to fissuring of the skin. Vulvar
fissures, spontaneous or secondary to physical
irritation and/or sexual intercourse, result on
dyspareunia and dysuria (Thorstensen and
Birenbaum 2012; Pugliese et al. 2007).
LS is in constant evolution affecting a small,
single area or spread to the entire vulva, perineum, and perianal skin. The characteristic anatomic sites affected by frequency are the
following: labia minora, labia majora, interlabial
sulci, clitoral hood, and perineum and perianal
areas (Simpkin and Oakley 2007
;Lorenzetal.
1998). This distribution can lead to the hallmark
presentation in “figure-of-eight.” Typical lesions
are porcelain-white papules and plaques with
follicular plugging. Hypopigmentation, sclerosis,andatrophyareseeninthesepatients.The
crinkling or cellophane paper type appearance is
the resul t of atrophy (Fig. 23). The fragility
N
Clinical Features
LS, chronic disease with insidious or aggressive
onset, has waxing and waning symptoms,
although some patients may remain asymptomatic
for long periods of time (Neill et al. 2010). Delay
in the diagnosis of this disease is reported to be
approximately 5 years, and it is due to the wide
range of signs and symptoms including vulvar
discomfort and pruritus (Cooper et al. 2004). LS
can lead to architectural disruption that often
results in reduced quality of life and deterioration
of sexual health. Vulvar pruritus, the most common symptom, worsens at night and may be
Non-neoplastic Lesions of the Vulva (Inflammations,
Dermatologic Conditions, Infections), Pathology
of the Vulva, Fig. 23 Hypopigmented, crinkled skin of
lichen sclerosus with well-demarcated macules and purpura
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suffer from constipation (Maronn and Esterly
2005). Purpura and fissures may mimic sexual
abuse (Furano 2004). LS does not exclude sexual
abuse as some cases of LS may be aggravated due
to Koebner phenomenon. Disease activity appears
to be reduced with puberty; however, it rarely
goes into complete remission. The incidence of
remission at puberty is estimated at 25% (Smith
and Fischer 2009).
LS is a clinical diagnosis, in most cases.
Anogenital itch associated with erythema and
white skin changes with crinkled texture should
arise suspicion of LS. The role of a biopsy in a
patient with clinically diagnosed LS without concern of malignancy is debatable. Notwithstanding
the different position s regarding the need of a
biopsy in a clinically evident LS, all guidelines
concur that these clinical scenarios should trigger
a vulvar biopsy (Edwards et al. 2015; van der
Meijden et al. 2017; Simpson et al. 2013):
1. Suspicion of malignancy: hyperkeratotic areas,
Non-neoplastic Lesions of the Vulva (Inflammations,
Dermatologic Conditions, Infections), Pathology
of the Vulva, Fig. 24 Well-demarcated white, shiny
plaque of lichen sclerosus, showing late scarring with
loss of the labia minora and puffiness and effacement of
the clitoral hood
new papules/nodules/plaques, hyperpigmentation, nonhealing ulcerated lesion, and lesions
with changes in vascular pattern.
2. Diagnostic uncertainty.
3. Failure of adequate treatment.
created by LS manifests as fissures, purpura, and
ecchymoses. LS is a scarring disease producing
agglutination of the labia minora, sealing of the
clitoral hood, and narrowing of the introitus
(Cooper et al. 2004;Fig.24). Marked
lichenification can be seen as result of the itchscratch cycle. Vaginal involvement is uncommon
in LS (Longinotti et al. 2005). Patients may have
extragenital lesions of LS, usually a symptomat ic
affecting the neck, shoulders, upper trunk, and
distal arms (Cooper et al. 2004; Simpkin and
Oakley 2007;Thomasetal.1996).
LS in prepubertal girls is similar to the adult
presentation with few differences. The most frequent presenting symptoms are anogenital itch,
irritation, and soreness. Fissures are associated
with symptoms including constipation and urinary symptoms (Cooper et al. 2004). In a study
of 18 girls with anogenital LS, 89% reported at
least one gastrointestinal complaint of whom 67%
An early diagnosis is essential for timeliness
and appropriate management that can halt the
repetitive cycles of damage and repair that leads
to disfiguration of the vulva and potential complication like vulvar squamous cell carcinoma.
• Incidence
The prevalence of LS is unknown; it is
suspected to be around 0.1–3% for pediatric
and postmenopausal women, respectively
(Kirtschig et al. 2017). These values may not
reflect the real incidence of the disease as
women may delay medical attention, asymp-
tomatic presentation, and misdiagnosed cases
(Neill et al. 2010).
• Age
There are two classical ages of onset: early
childhood and postmenopausal (Meffert et al.
1995; Fistarol and Itin 2013), but this condition
may start at any age.
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• Sex
Although it has a female predominance, it can
be observed in both sexes.
• Site
Typical anogenital region.
• Treatment
Treatment can control but not cure this disease.
Topical high potency steroid and calcineurin
inhibitors as steroid sparing thera py are frequent treatments in this disease. Surgery is
used to reduce strictures.
• Outcome
LS can be progressive or follow a relapsing
clinical course. There is no correlation between
the duration of the disease with the clinical
symptomatology and histopathological
changes (Marren et al. 1997; Fistarol and Itin
2013). In a study of newly diagnosed LS in
adults, 58% of patients were asymptomatic;
however, many present scarring of the clitoral
prepuce or resorption of the labia minora
(Goldstein et al. 2005). The majority of remissions occur in the first 3 months after treatment
(Rosamilia et al. 2006).
LS has been considered to carry an
increased risk for the development of vulvar
SCC in adults, driven by p53 mechanisms (van
Seters et al. 2007; Carlson et al. 1998). LS is
well known to be associated with dVIN and
keratinizing vulvar SCC. SCC is detected in
3.5–7% of patients with vulvar LS (Singh and
Ghatage 2020). The risk of neoplasia each year
is 1%, after 25 years, it can be 37% (Singh and
Ghatage 2020). In a study of 3038 women with
LS, the 20-year incidence of VSCC was
reported as 6.7% (Bleeker et al. 2016). The
mechanisms and real incidence of malignant
transformation of LS are subject to debate.
Some authors propose that only small proportion of the changes next to the SCC are typical
LS, whereas most cases correspond to differentiated vulvar intraepithelial neoplasia
(dVIN). Notably, allelic imbalance in the
same chromosomal loci of vulvar SCC has
been reported in 40% of LS in one study
(Pinto et al. 2000). Furthermore, the reported
rate of TP53 mutations in LS is of 6% (Trietsch
et al. 2015). Other factors proposed to promote
LS progression are hypermethylation of
MGMT and RASSF2A (DNA repair protein
and cell cycle regulator, respectively) detected
in LS associated with SCC (Guerrero et al.
2011). Global methylation and
hydroxymethylation aberrations are additional
epigenetic identified in LS progression
(Gambichler et al. 2014).
Macroscopy
Irregularly shaped white to erythematous plaques
with a tissue paper-like appearance.
Microscopy
LS is a member of lichenoid dermatoses characterized by pathologic alterations affecting the
three compartments of the skin or mucosa: squamous epithelium, basement membrane zone, and
dermis/submucosa.
Scurry and colleagues reported that genital LS
has a mean epidermal thickness greater than three
times that of extragenital LS (Scurry et al. 2001),
while extragenital LS has the tendency to be atrophic (Carlson et al. 1998). A well-developed
lesion of LS displays squamous epithelium with
hyperkeratosis, thinning with reduction of the rete
ridges, vacuolar alteration of the basal layer, and
rare dyskeratotic cells. These changes are associated with a broad zone of subepidermal edema
with homogenization of collagen or sclerosis and
a band-like lymphocytic infi
homogenized zone (Fig. 25a, b). Genital LS,
unlike nongenital lesions, often has absence of
epidermal atrophy with epithelium ranging from
normal to acanthotic. These patients suffer from
itch; therefore, superimposed changes of lichen
simplex chronicus in the form of acanthosis,
hypergranulosis, hyperkeratosis, and dermal
fibrosis are common (Carlson et al. 1998). Few
eosinophils may be seen as a part of the infiltrate.
Subepidermal edema occasionally leads to subepithelial bullae. Telangiectasias and subepidermal hemorrhagic bullae can also be seen.
Carlson and colleagues proposed that dermal
sclerosis along with interface changes is the minimum criteria for the diagnosis of LS (Carlson
et al. 1998). If only characteristic features are
used to identify LS, numerous cases would be
ltrate underneath the
N
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