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440 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. 25 Histologically, a well-developed
lesion of LS displays squamous epithelium with hyperkeratosis, vacuolar alteration of the basal layer, and rare
overlooked. A constellation of histologic features
is reported as useful in this challenge, and their
critical use may increase the early diagnosis of the
disease. We also need to keep in mind that LS is a
dynamic process, and a good correlation between
the disease duration and histologic features is not
always clear (Marren et al. 1997) as demonstrated
by a study with 33.3% of biopsies from patients
with classical clinical presentation of LS failing to
show definitive histologic features (McCarthy
et al. 2019). Thus, if the diagnosis is not certain
at the time of the biopsy, classification by inflammatory pattern like lichenoid/interface dermatitis
may be considered to prevent labeling the patient
with the incorrect diagnosis.
Squamous acanthosis/hyperplasia might present, previous to the development of sclerosis.
Weyers recognized a unique constellation of findings in his study of 100 cases of LS in search of
clues to facilitate the diagnosis of the
psoriasiform/lichenoid stage of LS. This consists
of vertical columns of parakeratosis overlying
psoriasiform epidermal hyperplasia and presence
of numerous necrotic keratinocytes clustered
above elongated dermal papillae (Fig. 26). Basement membrane (BM) may show changes at the
onset of LS; the most frequent alteration consists
dyskeratotic cells. These changes are associated with a
broad zone of subepidermal edema (a) with homogenization of collagen or sclerosis (b) and a band-like lymphocytic infiltrate underneath the homogenized zone
of an increase in thickness, highlighted by periodic acid shift (PAS) stain (Mann and Cowan
1973).
One of the first dermal change observed in LS
is the collection of protein-rich edema
(Montgomery and Hill 1940). Hyalinization is
the result of modulation of fibroblast activity and
collagen homeostasis ending in deposition of type
V collagen, dehydration, and deposition of fibrin
(Godoy et al. 2015; Bushkell et al. 1981; Tran
et al. 2019). These findings can be seen in association with vascular ectasia and sometimes
hyalinized wall (Fig. 27). These vascular changes
have been identified as the origin of clinical erythema and edema seen in LS in the absence of
sclerosis (Regauer et al. 2005). Fu rthermore, petechia has been related to the presence of lymphocytic vasculitis (Regauer et al. 2004). The
inflammatory infiltrate, first with a perivascular
distribution, consists primarily of lymphocytes
or a mixture of lymphocytes and histiocytes.
Over time, the inflammation will acquire a
band-like distribution with direct apposition to
the squamous epithelium. When the inflammatory
infiltrate approaches the epidermis, it tags along
the basal layer and lymphocytic exocytosis with
peppering of the entire epidermis can be observed
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Non-neoplastic Lesions of the Vulva (Inflammations,
Dermatologic Conditions, Infections), Pathology
of the Vulva, Fig. 26 In LS in the absence of sclerosis,
vertical columns of parakeratosis overlying psoriasiform
epidermal hyperplasia and presence of numerous necrotic
keratinocytes clustered (arrows) above elongated dermal
papillae can be seen
(Fig. 28). In a review of 71 cases of LS, wiry
fibrosis arranged parallel to the surface, with lymphocyte entrapment in single-filing distribution,
was also proposed as dermal change (Chan and
Zimarowski 2015; Weyers 2015) (Fig. 29).
Eosinophils in tissue is a relatively common
finding in LS. It h as been shown in a range of
29–65% of cases (Carlson et al. 1998;Lester
and Swick 2014; Keith et al. 2015). A study by
Keith et al. fails to show statistical significance
between eosinophils in LS and diseases associated with tissue eosinophilia like allergic contact dermatitis or autoimmune bullous diseases
(Keith et al. 2015).
The potential role of elastic fiber alterations in
the diagnosis of nonsclerotic LS has been a point of
contingency. Fung and colleagues demonstrated a
marked decreased in oxytalan fibers in all studied
cases of LS without sclerosis, concluding that elastic stain may be a useful diagnostic tool (Fung and
Non-neoplastic Lesions of the Vulva (Inflammations,
Dermatologic Conditions, Infections), Pathology
of the Vulva, Fig. 27 In LS in the absence of sclerosis,
vascular ectasia and sometimes hyalinized wall and thickening of the basement membrane can be identified
Non-neoplastic Lesions of the Vulva (Inflammations,
Dermatologic Conditions, Infections), Pathology
of the Vulva, Fig. 28 When the inflammatory infiltrate
approaches the epidermis, it tags along the basal layer, and
lymphocytic exocytosis with peppering of the entire epidermis can be observed
LeBoit 1998). Mihara et al. revealed that elastin
elaunin fibers in early LS were normal (Mihara
et al. 1994). The limited diagnostic value of elastic
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Morphea, localized presentation of scleroderma that affects primarily the skin, shares with
LS the homogenization of collagen fibers; however, morphea fails to show the crinkling or cellophane paper type appearance as the result of
atrophy as seen in LS. The thickened collagen
bundles generally present in morphea involve
the entire reticular dermis without basal vacuolar
alterations as opposed to the superficial location
of changes of LS. Preservation of superficial elastic tissue is characteristic of morphea as well as
peri-eccrine chronic inflammation with abundant
plasma cells. In late-stage radiation dermatitis,
Non-neoplastic Lesions of the Vulva (Inflammations,
Dermatologic Conditions, Infections), Pathology
of the Vulva, Fig. 29 Wiry fibrosis arranged parallel to
the surface, with lymphocyte entrapment in single-filing
distribution, was also proposed as dermal change in LS
previous to the deposition of sclerosis
atypical endothelial cells and fibroblasts and perivascular fibrin deposition can help to differentiate
this disease over LS. Sclerotic of collagen with
admixed elastotic material is also a helpful clue
favoring radiation dermatitis. Of paramount
important is the clinical history of this type of
fiber alteration was confirmed by Weyers; in his
study of 29 of LS without sclerosis, three quarters
of the cases fail to show abnormalities of elastic
fibers (Weyers 2015).
Changes in the adnexal structures can be seen
even before the epidermal alterations can be
detected. Squamous acanthosis associated with
hypergranulosis and hyperkeratosis of the infundibulum and acrosyringium are among the earliest
findings seen in the folliculo-sebaceous unit and
sweat glands, respectively (Regauer et al. 2005;
Fung and LeBoit 1998).
treatment.
Part of the differential diagnosis of LS without
sclerosis includes contact dermatitis that may be
allergic or irritant in nature, affecting approximately 15–30% of the women. The presence of
necrotic keratinocytes with associated neutrophils
suggests irritant contact dermatitis (Kanerva
1990; Willis et al. 1989), while Langerhans cell
microabscess in association with eosinophils in
the dermal/submucosal infiltrate favors allergic
contact dermatitis (Bowen et al. 2008). In subacute to chronic phase of these diseases, the epidermis becomes less spongiotic and acquires a
Differential Diagnosis
The differential diagnosis differs if sclerosis is present in the biopsy. If present, sclerotic process like
morphea and late-stage radiation dermatitis should
be considered. A biopsy of LS with an inflammatory
presentation and absence of sclerosis needs to be
differentiated to a range of disorder that can be
divided in two groups based on the presence of
squamous acanthosis. If squamous acanthosis is
present, the differential diagnosis include s subacute
to chronic contact dermatitis, candidiasis, psoriasis,
and hypertrophic lichen planus. A sample without
acanthosis raises the differential dia gnosis of lichen
planus, erythema multiforme, and fixed drug
reaction.
psoriasiform hyperplasia, overlapping with those
seen in LS in the absence of sclerosis. Vacuolar
changes at the dermal epidermal junction in association with basal dyskeratotic cells, thickened
BM, and absence of perpendicular fibrosis can
be useful clues to favor LS over subacute to
chronic phase of eczematous dermatitis.
Although LS without sclerosis and candidiasis
have in common psoriasiform spongiotic epidermal acanthosis, the presence of microorganisms
and the presence of neutrophils can separate both
disorders.
Psoriatic lesions observed in the labia majora
and intertriginous areas will display not only
psoriasiform spongiotic hyperplasia with club-
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shaped rete ridges but also hypogranulosis, thinning of the suprapapillary plate, neutrophilic
spongiosis, and dermal papillae capillary ectasia
(Chau et al. 2017). These findings are not seen in
LS with absence of sclerosis.
Lichen planus (LP), a papulosquamous disease
that affects mucocutaneous surfaces, shares with
nonsclerotic LS the presence of a band-like lymphoid infiltrate, vacuolar alteration of the basal
layer, and the presence of basal dyskeratotic
cells. Vulvar LP has been grouped in three categories: typical LP as seen in extragenital skin,
erosive LP, and hypertrophic LP (Day et al.
2020). Due to the wide range of LP presentations,
it is in the differential diagnosis of LS with or
without epidermal hyperplasia. The degree of
basal layer damaged in the form of
squamatization, degree of vacuolar changes, and
cytoid bodies is more pronounced in LP over
nonsclerotic LS. The challenge is more considerable when LP presents predominantly in mucosa
or modified mucosa, which is erosive LP. The
degree of squamatization of the basal layer can
be used to support the interpretation of LP over
nonsclerotic LS. Fung and Weyers also found the
presence of cytoid bodies a distinguishable feature commonly seen in LP. Dyskeratotic cells will
not drop down in the dermis to create cytoi d
bodies in LS; they may be numerous with the
tendency to be seen in the upper portion of the
squamous epithelium. In keeping with the tendency to see more evidence of basal layer damage
with numerous dyskeratotic keratinocytes in LP,
numerous melanophages point to LP over
LS. Hypertrophic LP and nonsclerotic LS will
show squamous acanthosis/hyperplasia with
hyperkeratosis; however, the presence of
psoriasiform epidermal hyperplasia with zones
of parakeratosis and spongiosis is rare in LP and
can be considered clues to favor LS with no sclerosis, especially in conjunction with lymphocytes
aligning the basal layer or peppering the squamous epithelium at different levels (Fung and
LeBoit 1998; Weyers 2015).
Erythema multiforme and fixed drug eruptions
share with nonsclerotic LS the presence of
dyskeratotic cells at different levels of the
squamous epithelium or in clusters, although
dyskeratosis is more prominent inerythema multiforme or fixed drug eruption.
It is also important to differentiate LS from
vulvar verruciform lesion and differentiated vulvar intraepithelial neoplasm (dVIN). There is a
group of verruciform lesions that includes vulvar
acanthosis with altered differentiation (VAAD),
verruciform LSC, and atypical verruciform
hyperplasia. These lesions share verruciform
growth pattern, abnormal keratinocytic differentiation, and absence of significant basal atypia. In
addition, the genetic landscapes are characterized by absence of TP53 mutations and high
frequency of PIK3CA mutations. Therefore, the
name “differentiated exophytic vulvar
intraepithelial lesions” or DEVIL has been proposed to encompass these verruciform lesions
(Watkins et al. 2017).
Watson et al. point to the presence of
dyskeratotic cells in LS with a tendency to group
overlying a localized area of columnar parakeratosis as features not seen in VAAD with its
classic confluent plaque-like parakeratosis
(Walton et al. 2015). Verruciform LSC combines
acanthosis with variable verruciform architecture,
prominent granular cell layer and hyperkeratosis.
This entity is indistinguishable from conventional
LSC except by the presence of verruciform morphology. Vacuolar alteration of the basal layer and
presence of dyskeratotic cells as seen in LS are
notcomponents seen in these verruciform lesions.
dVIN and LS share the presence of elongation
of rete ridges. While the hyperplasia in nonsclerotic LS is psoriasiform (thick rete ridges
with round end and similar length), the epidermal
thickening of dVIN results from elongation,
branching, and anastomosis of rete ridges. In addition to the architectural abnormalities described in
dVIN, there is a disturbed maturation of
keratinocytes with deep kerat inization
(premature maturation) and squamous whorls formation not seen in LS. A significant degree of
nuclear atypia and increased mitotic activity seen
in dVIN are not characteristic of LS. Expression
of p53 in a confluent strong staining pattern with
suprabasal distribution or aberrant negative
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444 Non-neoplastic Lesions of the Vulva
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pattern would favor the diagnosis of dVIN (Heller
et al. 2021).
Lichen Simplex Chronicus
Definition
Lichen simplex chronicus (LSC) is a common cause
of vulvar itching characterized by the itch-scratch
cycle. Itching leads to chronic, excessive rubbing
and scratching causing skin thickening, which leads
to additional irritation and itching. Itching is often
severe and can be intractable despite treatment.
Symptoms often begin during times of stress and
can be worse at night. Other factors that can worsen
symptoms include heat, sweating, friction, vulvar
products, menses, urine, and stool.
Lichen simplex chronicus can represent either
a primary or secondary process. Primary lichen
simplex chronicus can be considered as a
chronic, localized form of atopic dermatitis. Personal or family history ofatopy is a risk factor for
primary lichen simplex chronicus. Up to 90% of
patients with vulvar lichen simplex chronicus
have a history of atopy; however, other studies
did not show an increased prevalence of atopy
over the general population. In secondary vulvar
lichen simplex chronicus, an underlying disorder
leads to the itch-scratch cycle. Associated disorders include contact dermatitis, psoriasis, lichen
sclerosus, candidiasis, tinea cruris, HPV, and
neoplasia.
• Treatment
Treatment of vulvar lichen simplex chronicus
involves a multifactorial approach to break the
itch-scratch cycle. Topical steroids are the most
common therapy employed and decreases vulvar
inflammation. Antihistamines can help relieve
itching. Patients should be educated to avoid
potential triggers like urine, fecal incontinence,
sweat, certain fabrics, and panty liners. Other
second-line therapies that have been used include
calcineurin inhibitors and topical anesthetics.
• Outcome
Lichen simplex chronicus is a chronic, relapsing condition, and symptoms may persist
despite removal of triggers. Following treatment, it is prudent to reexamine the patient to
exclude an underlying condition leading to
lichen simplex chronicus. There is no
increased risk of squamous cell carcinoma.
Macroscopy
On physical exam, lichen simplex chronicus is
characterized by licheni fied plaques with accentuation of skin markings (Fig. 30). Excoriation
leads to angular or linear erosions. Scale may be
present; however, this is a less consistent feature
in anogenital sites as compared to extragenital
sites of involvement due to the persistent moistness in anogenital areas. Variable erythema and
pigmentary alteration, which may be
Clinical Features
• Incidence
Incidence and prevalence of vulvar lichen sim-
plex chronicus have not been formally studied;
however, in vulvar speci alty clinics 10–35% of
patients present with lichen simplex chronicus.
• Age
Vulvar lichen simplex chronicus is primarily
seen in adults; however, it can rarely occur in
children.
• Site
The labia majora is the most common site
involved and may be unilateral or bilateral.
Other less common locations that can be
involved include the labia minora, perineum,
mons pubis, and inner thighs.
Non-neoplastic Lesions of the Vulva (Inflammations,
Dermatologic Conditions, Infections), Pathology
of the Vulva, Fig. 30 On physical exam, lichen simplex
chronicus is characterized by lichenified plaques with
accentuation of skin markings. (Courtesy of Dr. Libby
Edwards)
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Non-neoplastic Lesions of the Vulva (Inflammations,
Dermatologic Conditions, Infections), Pathology
of the Vulva, Fig. 32 Histologically, lichen simplex
chronicus includes irregular acanthosis with hyperkeratosis and hypergranulosis. Mild spongiosis and variable
superficial perivascular chronic inflammation are also seen
or candidiasis, can cause vulvar itching and
secondary lichen simplex chronicus. In office
potassium hydroxide (KOH), prep and periodic
Non-neoplastic Lesions of the Vulva (Inflammations,
Dermatologic Conditions, Infections), Pathology
of the Vulva, Fig. 31 Variable erythema and pigmentary
alteration, in this case hyperpigmentation, is noted in these
patients. (Courtesy of Dr. Libby Edwards)
acid-Schiff stain on histopathologic sections can
help exclude fungal infection. Psoriasis with
superimposed rubbing or partial treatment can
exhibit a prominent granular layer or spongiosis
and, thus, can be challenging to distinguish from
hyperpigmentation or hypopigmentation, can be
seen (Fig. 31).
vulvar lichen simplex chronicus. However, few
findings characteristic of psoriasis can help in
the diagnosis such as thinning of the supra-
Microscopy
Vulvar lichen simplex chronicus is similar histopathologically to lichen simplex chronicus in
other sites. Changes include irregular acanthosis
with hyperke ratosis and hypergranulosis. Mild
spongiosis and variable superficial perivascular
dermal chronic inflammation can be present. In
papillary plate, collections of neutrophils in the
stratum corneum as well as congested superficial
blood vessels. Clinicopathologic correlation is
necessary to adequately differentiate these two
entities with emphasis in identifying extragenital
manifestations of psoriasis in areas like scalp,
nails, and elbows.
contrast to extragenital lichen simplex chronicus,
vulvar LSC often lacks vertical papillary fibrosis
and is more likely to have prominent fibroblasts
Pemphigus
(Fig. 32).
Definition
Differential Diagnosis
In patients with vulvar lichen simplex chronicus,
it is important to exclude an underlying primary
dermatosis as the cause. Sometimes, it is necessary to treat the lichen simplex chronicus before
determining if there is an underlying primary vulvar dermatosis. Fungal infection, like tinea cruris
Pemphigus is an heterogenous group of acquired
immunobullous disorders affecting adhesion molecules in cutaneous and mucosal surfaces.
Decohesion of keratinocytes results in
acantholysis and blister formation. Pemphigus
vulgaris and pemphigus foliaceous are mediated
by autoantibodies against desmoglein 3 and
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446 Non-neoplastic Lesions of the Vulva
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desmoglein 1, respectively. Endemic pemphigus
foliaceous also known as fogo selvagem is
believed to be triggered by environmental antigens that mimic desmoglein 1.
Clinical Features
Vulva is the second most frequent site of mucosal
involvement in patient suffering from pemphigus
vulgaris, with a reported frequency of 22–51 %
(Akhyani et al. 200 8 ;Curaetal.2020). Rarely
genital lesion can be the only manifestation of
the d isorder; this occurs especially in young
adults (Malik and Ahmed 2005). In the two variants described in children, childhood, and juvenile types, genital involvement is more frequent
when compared to the adult presentation. Beside
the vulva, the uterine cervix and vagina are also
affected in this disease (Batta et al. 1999). The
characteristic clinical presentation of vulvar
pemphigus vulgaris consists of painful flaccid
blisters that rapidly turn into well-demarcated
erosions (Fig. 33). Genital lesions are c ommonly
accompanied by oral and nasal involvement
(Kavala et al. 2015). The extragenital lesions
are characterized by round to oval bullae in nonerythematousbasedwithpredilectiontoaffect
scalp, face, axillae, and groins. Nikolsky’ssign
Non-neoplastic Lesions of the Vulva (Inflammations,
Dermatologic Conditions, Infections), Pathology
of the Vulva, Fig. 33 The extreme fragility of pemphi-
gus vulgaris can produce poorly demarcated erosions that
belie the underlying blistering nature of the disease.
(Courtesy of Dr. Libby Edwards)
is present. A vegetative localized variant of pemphigus vulgaris or pemphigus vegetans presents
with warty or hypertrophic projections that can
be associated with bullae in perianal, inguinal
region, mammary and axillary f olds, and scalp
(Zaraa et al. 2011;Ajbanietal.2019 ). Pemphigus foliaceous presents with recurrent crops off
flaccid bullae that rupture resulting in crusted
plaques on skin with sparing of mucosal surfaces, as the autoantibody of this variant of pemphigus or desmoglein 1 concentrations is lower
in mucosa. A series from Brazil documented
genital involvement by pemphigus foliaceus in
27% of patients (Fairbanks Barbosa et al. 2012).
Rarely the clinical, histologic, and antibody profile may change from pemphigus vulgaris to
pemphigus foliaceus.
vegetans. The overall incidence of pemphigus
vulgaris is 0.1–3.2 per 100,000 with reported
vulvar involvement that range from 22 to 51%
(Kavala et al. 2015).
• Age
Patients affected with this disease are frequently between the fifth and eighth decade.
Pediatric cases have been reported. Onset of
pemphigus vulgaris before the age of 40 years
has been proposed as a sign of poor outcome as
these patients have a tendency of suffering
from generalized skin involvement (Cura
et al. 2020).
• Sex
Female.
• Site
Vulva is the most frequent organ involved when
• Incidence
Pemphigus vulgaris is the most frequently type
seen in genitalia followed by foliaceous and
pemphigus vulgaris affects the genitals. Genital
areas involved, in order of frequency, are labia
majora and minora followed by vagina.
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Non-neoplastic Lesions of the Vulva (Inflammations,
Dermatologic Conditions, Infections), Pathology
of the Vulva, Fig. 34 In pemphigus vulgaris,
• Treatment
Topical or systemic immunosuppressants are
the treatment of choice, depending on the
extent of the disease.
• Outcome
Pemphigus vulgaris in 34% of patients pre-
sent with recurrent flares. The involvement of
the vulva in this disease implies aggressive
course with fatalities mainly due to secondary
infections or fluid loss. Pemphi g us vegetans
has in general a less aggressive course as it
rarely has a widespread presentation. Pemphi-
gus foliaceous is characterized by an indolent
course. An isolated case of squamous cell
carcinoma has been reported in patient suffer-
ing from pemphigus vulgaris (Bifulco
et al. 2010).
Macroscopy
Flaccid blisters and well-delineated erythematous
erosions.
Microscopy
In pemphigus vulgaris, acantholysi s leads to a
suprabasal blister with basal keratinocytes
aligned at the floor of the blister mimicking a
row of “tombstones” (Fig. 34a, b). The roof of
the blister shows scalloping off keratinocytes
due to acantholysis; no dyskeratotic cells are
seen. These changes involve adnexa l structures
like hair follicles (Fig. 35). A moderately dense
acantholysis leads to a suprabasal blister (a) with basal
keratinocytes aligned at the floor of the blister mimicking
a row of “tombstones” (b)
N
Non-neoplastic Lesions of the Vulva (Inflammations,
Dermatologic Conditions, Infections), Pathology
of the Vulva, Fig. 35 The acantholysis seen in pemphi-
gus vulgaris can involve adnexal structures like hair follicles (arrow)
mixed inflammatory infiltrate composed of lymphocytes, neutrophils, and few eos inophils
canbeseenaroundvesselsandinthe
interstitium of the superficial dermis. The distinctive feature of pemphigus foliaceus is the
presence of a subcorneal blister with
dyskeratotic granular layer cells (Fig. 36). Pemphigus vegetans is characterized by verruco us
epidermal acanthosis with presence of
intraepidermal microabscesses with acantholytic cells, eosinophils, and few neutrophils
(Fig. 37). Like in pemphigus vulgaris, involvement of adnexal structures can be seen.
448 Non-neoplastic Lesions of the Vulva
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Immunophenotype
For all three types of pemphigus, direct immunofluorescence shows the deposition of intercellular
IgG plus minor C3 with a lace-like pattern. Circulating autoantibodies to epidermal intracellular
spaces can be identified with indirect immunofluorescence methods against monkey esophageal
epithelium. These titters correlate with disease
activity in patients. The possibility to use plucked
hair follicles to run direct immunofluorescence
test has been recently reported.
Differential Diagnosis
Acantholytic disorders like Hailey-Hailey disease, Darier’s disease, and papular acantholytic
Non-neoplastic Lesions of the Vulva (Inflammations,
Dermatologic Conditions, Infections), Pathology
of the Vulva, Fig. 36 The distinctive feature of pemphi-
gus foliaceus is the presence of a subcorneal blister with
dyskeratotic granular cells
dyskeratosis of the v ulvocrural area are part of
the differential diagnosis of pemphigus
vulgaris. Immunofluorescent studies are negative in these entities, making this test a
useful diagnostic tool. Follicular involvement
is not seen in these acantholytic disorders
supporting pem phigus vulgaris, while
dyskeratotic cells a re not a feature of pemphigus
vulgaris. Marked dermal inflammatory reaction
is characteristic of Hailey-Hailey disease in
contrast to the pauci-inflammatory reaction
seen in pemphigus vulgaris. Infection is in
the differential diagnosis of pemphigus
vegetans due to the marked epidermal hyperplasia and inflammation. Special stains for microorganisms and immunofluorescent tests are
helpful in this differential diagnosis. Although
bullous impetigo, dermatophytosis, and staphylococcal scalded s kin syndrome share some histologic features with pemphigus foliaceus,
a positive direct immunofluo rescent test
result will be able to separate pemphigus
foliaceus from these diseases. In addition,
Gram and periodic acid-Schiff (PAS) stains
can h ighlight the presence of intracorneal bacterial and fungal forms in bullous impetigo and
dermatophytosis, respectively. Intercellular IgA
deposition of direct immunofluorescenttestcan
separate IgA pemphigus from pemphigus
foliaceous.
Non-neoplastic Lesions of the Vulva (Inflammations,
Dermatologic Conditions, Infections), Pathology
of the Vulva, Fig. 37 Pemphigus vegetans is
characterized by verrucous epidermal acanthosis (a) with
suprabasal acantholysis (b) and presence of intraepidermal
microabscesses containing eosinophils and neutrophils (c)
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Plasma Cell Vulvitis
Synonyms
Plasmacytosis mucosae; Vulvitis circumscripta
plasmacellularis; Zoon vulvitis.
Definition
Plasma cell vulvitis is an idiopathic plasma cellrich inflammatory disease with a distinctive clinical presentation.
Clinical Features
The pathogenesis of this disease is unknown;
however, factors that may play a role in this disease include trauma, infection, autoimmune
response to an unknown antigen, and leaking vessels (Li et al. 2003). Plasma cell mucositis can
affect other organs like oral cavity and the glans
penis. The clinical presentation is characterized
by a solitary sharply defined red brown patch.
Dyspareunia, dysuria, pruritus, or pain are common complaints in these patients (Virgili et al.
2015). In children, this disease may be confused
with child abuse (Albers et al. 2000), a possibility
that should never be disregarded without meticulous investigation. Rare cases of plasma cell
vulvitis has been associated with autoimmune
polyglandular syndrome (Salopek and Siminoski
1996), lichen sclerosus (van Kessel et al. 2010),
acquired immunodeficiency syndrome (AIDS),
and herpes simplex virus type II infection
(Kuniyuki et al. 1998; Elghobashy et al. 2020).
involved (Neri et al. 1995; Mauskar
et al. 2020).
• Treatment
The treatment ranges from ultrapotent topical
corticosteroid ointments (e.g., clobetasol or
halobetasol) to intralesional triamcinolone
acetonide if there is no improvement.
Tacrolimus ointment, pimecrolimus cream,
and CO
laser has been proposed for cases
2
not responding to first-line treatment. This disease moderately responds to treatment and
rarely resolves.
• Outcome
The disease has a benign but chronic course
and is recalcitrant to therapy with a minor
percentage of the patients showing consistently
satisfactory results to treatment (Toeima et al.
2011). Patients suffering from plasma cell
vulvitis do not carry an increased risk of
vulva squamous cell carcinoma.
Macroscopy
Plasma cell vulvitis presents as a welldemarcated, shiny, red to rusty patch or plaque
with rare ulceration (Fig. 38). The size of the
primary lesion varies from 1 to 3 cm. Usually
present as a single lesion, however, several confluent macules have been described.
N
• Incidence
Due to the low frequency of the disease, its
incidence has not been determined.
• Age
It is a disease affecting adult women (Neri et al.
1995; Pourzan and Laird-Fick 2019) with rare
cases reported in prepubertal girls (Albers
et al. 2000).
• Sex
Female.
• Site
The classic location of plasma cell vulvitis is
the vestibule and periurethral area with exten-
sion to the labia minora. The clitori s is rarely
Non-neoplastic Lesions of the Vulva (Inflammations,
Dermatologic Conditions, Infections), Pathology
of the Vulva, Fig. 38 Orange-red patches of plasma
cell vulvitis on mucous membrane skin that show the
rusty appearance. (Courtesy of Dr. Libby Edwards)
Соседние файлы в папке Библиотека им академика М.И. Перельмана