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REVIEW

Skin diseases affecting


the vulva

Acute or chronic candidiasis should respond to imidazoles,


either intravaginally as pessary or cream, or orally. Persistent
symptoms can develop if only external treatment is used.
Recurrent episodes may respond best to a 7e10 day course of
oral fluconazole.

Moumita Chattopadhyay
Jane Sterling

Tinea cruris
Ringworm of the groin is another readily diagnosable and easilytreated cause of vulval irritation. A pre-existing problem with
athletes foot (tinea pedis) may lead to the suspicion of involvement of the groin and vulval skin. In the groin, buttocks and upper
thighs, fungal infection causes mild erythema with a well-defined
edge with fine scaling, but in hair-bearing areas, there may be
deeper involvement of the follicles with pustules or nodules. Skin
scrapings to capture fine scales for fungal culture can reveal the
causative agent, most commonly Trichophyton rubrum. If extensive, systemic therapy with terbinafine is most effective.
Easily confused with tinea is the condition Erythrasma,
caused by corynebacteria. The infection is more common in
immunosuppression or diabetes. A faint brownish discolouration
of the groin with fine peeling should suggest the diagnosis and, if
available, examination with Woods light (low wave length
ultraviolet) will show coral-pink fluorescence of affected skin.

Abstract
The vulva stretches between the mons pubis and the anus and is bounded
by the genitocrural folds. The epithelium includes the keratinized, hair-bearing squamous area of the labia majora and mons pubis and the squamous
mucosa of the vaginal introitus. The anatomy produces relative occlusion
of the area and contributes to high humidity and levels of surface organisms, whist hormonal variation also influences skin and mucosal function.
Skin disorders that can affect any part of the skin can appear slightly
different in the vulval area and there are a number of disorders that
occur more frequently at anogenital sites than elsewhere on the body.

Keywords blister; infection; inflammation; pruritus vulvae; skin; ulcer;


vulva; vulvar diseases; vulvar neoplasms

Presentation of vulval disease


Patients with vulval disease are often embarrassed by their
problem and may delay seeking help or self-treat. Careful elucidation of the symptoms and signs of vulval disease can be
sufficient to make the diagnosis (Table 1), but investigations
such as vaginal swabs, allergy patch testing and skin biopsy are
often needed to confirm suspicions or distinguish between
diseases with similar presentations.
A shortlist of the most common vulval conditions would
include vulvovaginal candidiasis, dermatitis and lichen sclerosus,
but there are many other less common causes of vulval symptoms
which must not be forgotten. It is always worthwhile considering
infection as a cause of or an exacerbating factor in vulval skin
disease.

Symptom

Itch

Discomfort/pain

Infections
Candida
A sensation of itch, both in the vagina and in the vulval skin,
combined with an increase in creamy vaginal discharge are the
hallmarks of vulvovaginal candidiasis. Treatment should be
preceded by vaginal swab to confirm the presence of the yeast.
Chronic or incompletely treated candidiasis may present with
persistent vulval erythema and fissuring in the interlabial sulci or
perineum. Again, culture will help to confirm the diagnosis.

Swelling
of vulva

Lump(s)
Moumita Chattopadhyay MB BS DDVL MRCP is a Specialist Registrar at the
Department of Dermatology, Addenbrookes Hospital, Cambridge, UK.
Conflicts of interest: none declared.

Blisters
Ulcers

Jane Sterling MB BChir MA FRCP PhD FHEA is a Consultant at the Department


of Dermatology, Addenbrookes Hospital, Cambridge, UK. Conflicts of
interest: none declared.

OBSTETRICS, GYNAECOLOGY AND REPRODUCTIVE MEDICINE 21:6

Possible diagnosis
Acute
Chronic continuous/
presentation
intermittent
Candida
Lichen simplex
Contact
Lichen sclerosus
dermatitis
Lichen planus
Tinea cruris (ringworm)
Partially controlled
contact dermatitis
Incompletely treated candida
Irritant
Psoriasis
dermatitis
Seborrhoeic dermatitis
Folliculitis
Intertrigo
Cellulitis
Erythrasma
Boils
Lichen sclerosus
Lichen planus
Plasma cell vulvitis
Vulvodynia/vestibulodynia
Cellulitis
Urticaria
Hidradenitis suppuritiva
Crohns disease
Lymphoedema
(primary or secondary)
Cysts
Warts
Hidradenitis suppuritiva
Molluscum
contagiosum
Malignancy
Herpes simplex
Pemphigus
(vulgaris or foliaceous)
Aphthous ulcers Nicorandil-induced ulceration
Behcets disease

Table 1

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REVIEW

Treatment for 2 weeks with topical fusidic acid or an imidazole


cream is usually effective.

twice daily for 6 months) can help to reduce the number of


reactivation episodes.

Folliculitis/boils
Folliculitis is visible as small papules and pustules sited at hair
follicles. Careful swabbing, if possible from the pus of an intact
pustule, is likely to show infection with Staphylococcus aureus, but
other aerobes and anaerobes can occasionally cause this common
infection. Damage to the skin through shaving presents with
a similar picture, but the infection may be more superficial without
deep involvement of the follicles and so is termed pseudofolliculitis.
The infection usually responds to a combination of topical
antiseptic measures and systemic antibiotics. Although the bacteria
are usually sensitive to flucloxacillin and erythromycin as well as
other anti-staphylococcal drugs, recurrence is common unless the
course of treatment is prolonged. General measures to reduce
surface bacteria by washing and antiseptic applications plus
increased ventilation to the area (weight loss if the patient is obese,
reduced sitting and loose-fitting, natural fibre clothing) will all help
to minimize infection recurrence.
Occasionally infection from an infected follicle can burst into
the adjacent subcutaneous tissue forming a larger inflammatory
mass with collection of pus recognizable as a boil or furuncle.

Molluscum contagiosum
Infection with this molluscipox virus produces small pearly
papules with a central dip or umbilication. In children, infection
is common on the trunk and limbs, but in young adults, genital
infection is more common. Clearance is usually spontaneous, but
can be hastened with cryotherapy.
Warts
Anogenital warts in adults are usually sexually transmitted and are
caused by the low-risk human papillomaviruses (HPVs), usually
type 6 or 11. The diagnosis is suggested by small papules or
cauliflower-shaped growths at any site in the vulva or vagina, but
the most common site is at the posterior fourchette.
The differential diagnosis includes molluscum contagiosum,
epidermoid cysts, lymphangiomata, angiokeratomas, seborrhoeic
keratoses and vulval intraepithelial neoplasia. If the diagnosis is in
doubt, histology and possibly also molecular testing for HPVs
should be obtained.
Treatment of vulval warts will depend on the number, size and
position of the warts. In immunosuppressed individuals, standard
therapies may not lead to clearance, as an immune response
against the virus is essential to clear the infection. Topical antiproliferative or destructive treatments are used most commonly
and include podophyllotoxin, trichloroacetic acid and liquid
nitrogen cryotherapy. The immune response modifier, imiquimod,
is also effective and can reduce the risk of recurrence. Warts that
are keratinized may not respond so well as the treatment may not
penetrate and in this case, surgery with snip or curettage may be
the treatment of choice.
Vulval warts can be associated with acquisition of the other
HPV types that are associated with cervical disease, so cervical
smear testing should not be forgotten.

Hidradenitis suppuritiva
Recurrent boils in the groin plus other flexural sites such as
axillae and sub-mammary areas together with comedones and
bridged scarring suggests the condition hidradenitis suppuritiva,
which may be familial.
Cellulitis
Streptococcal infection of the vulva presents with aching pain,
tenderness and a beefy-red swelling of the labia. There may be
associated increase in vaginal discharge and general symptoms of
malaise and fever. Culture may confirm the organism but treatment
should be started immediately to reduce the risk of secondary
lymphatic damage and hence increased risk of repeated episodes
and development of lymphoedema. Penicillin, flucloxacillin or
erythromycin are appropriate choices for oral antibiotics.

Disorders associated with malignancy


Vulval intraepithelial neoplasia (VIN)
Vulval intraepithelial neoplasia, like intraepithelial neoplasia of
the cervix, vagina, penis and perianal skin, is closely associated
with high-risk human papillomavirus infection, most commonly
HPV type 16. The presentation of VIN is varied. There may be no
symptoms but a change in surface texture or a change in
appearance may be noted either by the patient or at examination,
for instance at cervical screening. When present, symptoms
include irritation, mild discomfort, pain or ulceration. Visibly VIN
can appear as an area of glazed erythema, as an area of slightly
thickened, macerated skin, as an area of brown pigmentation or
a combination of any of these (Figure 1). VIN is more common in
patients with a history of cervical intraepithelial neoplasia.
If VIN is suspected, tissue diagnosis is essential. There are two
main histological types of VIN e well differentiated VIN and
basaloid VIN. The latter is more common, especially in the premenopausal woman.
The main cause for concern is the risk of progression to invasive squamous cell carcinoma (SCC), which is of the order of
about 5% over lifetime. Unifocal disease may be best treated
surgically and as long as the resection margins are clear, recurrence is unlikely. If margins are involved, recurrence is likely.

Crohns disease
Vulval Crohns disease has many features similar to those of
chronic cellulitis or hidradenitis suppuritiva. It may develop
before any intestinal disease or together with bowel Crohns.
Pustules, ulcers, fissures, sinuses, fistulae, papules, nodules or
skin tags with inflammation, swelling and induration can all
feature as part of this granulomatous inflammation. If suspected,
a search for bowel abnormality is essential.
Herpes simplex
Genital herpes is not uncommon and is usually diagnosed in
general practice or in genitourinary medical clinics. In primary
genital herpes, the onset is acute and the pain is severe. There
may be associated general malaise. Reactivation episodes are less
severe and more commonly present with pain plus a small group
of erosions and possibly short-lived blisters amongst them. Aciclovir by mouth will help to shorten the duration of the acute
eruption provided it is taken early in the course of the infection.
If episodes are frequent, regular prophylactic aciclovir (200 mg

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Figure 2 Pagets disease, extramammary.

Figure 1 Vulval intraepithelial neoplasia.

condition is asymptomatic. Inspection of the skin will show welldefined areas of whitening or at least some smoothing and shininess of the affected areas (Figure 3). The classical figure of eight
involvement of the vulva, perineum and perianal skin is not
always evident but within this region, the most commonly affected
sites are the clitoral hood and clitoris, the labia minora and
interlabial sulci and the perineum. The skin changes may include
bruising (ecchymoses), telangiectasia, thickening and hyperkeratosis, fissuring as well as ulceration. The vaginal mucosa is not
involved but loss of elasticity and sclerotic change at the vaginal
introitus can make intercourse painful or impossible. This scarring
process which arises as a result of long-standing inflammation
with damage to the dermis and dermoepidermal junction, can also
cause clitoral hood fusion with burying of the clitoris, resorption
of the labia minora, anterior or posterior fourchette fusion and
occasionally fusion of the labia minora with loss of the introitus.
Extragenital LS is unusual, causing patches of white shiny skin,
most commonly found on the back, abdomen or thighs.
Lichen sclerosus is an autoimmune disease and is associated
with other autoimmune disorders especially thyroid disease,
certain HLA antigens and circulating antibodies against extracellular matrix proteins. The major peak of presentation is postmenopausal, but pre-pubertal girls also show a smaller peak of
incidence. The risk of development of vulval squamous cell
carcinoma is approximately 5%.
The inflammatory process can be controlled with an ultrapotent topical corticosteroid. Clobetasol propionate 0.05% is the
mainstay of treatment in the UK, but other potent steroid preparations may be sufficient. After regular daily use for a month,
frequency of application can be reduced, but as the inflammation

Multifocal or recurrent disease presents greater problems as


surgery could be mutilating to remove all disease with an
adequate margin. Alternative treatments include laser and topical
therapies which can produce good results. Imiquimod cream and
cidofovir have both been reported to clear VIN, but the treatment
induces an intense inflammatory reaction with discomfort and
occasionally ulceration.
Extramammary Pagets disease
Extramammary Pagets disease can be an indication of an adjacent
or close adenocarcinoma, so a careful search for lower bowel or
gynaecological malignancy is essential. However, it can arise
without any internal cancer. The patient may have no symptoms,
but usually the affected skin is sore and the appearances are of
thickened erythematous skin with a macerated and eroded surface
(Figure 2). The biopsy features are mucin-containing cells within
the epidermis and a variable degree of surface erosion and dermal
inflammatory cell infiltrate. Treatment of vulval Pagets disease is
difficult. As in VIN, large areas of skin may be involved, making
surgery to clear disease potentially extensive. Laser ablation of the
skin can help symptomatically but does not treat deeply enough to
eradicate disease in follicular epidermis, so recurrence is expected.
Topical treatment with imiquimod has given good results in some
cases but does not work for all. In many cases, the disease remains
static and observation only can be considered.
Lichen sclerosus (LS)
Early symptoms of lichen sclerosus can be very similar to those of
candidiasis with itch being the predominant feature. Soreness and
dyspareunia are common complaints but occasionally the

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Figure 4 Lichen planus, papulosquamous form.

Figure 3 Lichen sclerosus.

squamous cell carcinoma is rare, but can occur, especially in


chronic erosive LP.
Treatment with bland emollients helps to reduce symptoms,
but local or systemic immunosuppression is necessary to reverse
the disease process. High-potency topical corticosteroids (usually
as ointments or suppositories, and sometimes intralesionally) are
the mainstay, with short-courses of oral steroids for flares. For
severe, recalcitrant disease, steroid-sparing immunosuppressants
such as ciclosporin, tacrolimus, methotrexate or azathioprine
may be necessary. Vaginal dilators can be used to treat or
prevent introital narrowing. Surgery should be avoided because
of disease activation in scar sites and the risk of developing
postoperative synechiae.

is chronic, treatment may also need to be long-term. Many


women can maintain adequate control with a once or twice
weekly application of topical steroid cream or ointment.
Concomitant usage of emollients is beneficial. Topical calcineurin inhibitors (e.g. tacrolimus) can be an alternative secondline treatment. Surgery is contraindicated as it can induce spread
of the disease (Koebnerization) but is essential if invasive SCC
develops and may be necessary if pre-malignancy is suspected or
scarring and fusion impede micturition.
Lichen planus (LP)
Lichen planus is an inflammatory condition of unknown cause. It
is most common in the oral mucosa, but also affects other
mucosal sites, external genitalia, skin, nails and hair. In women
with oral disease, over 50% may have vulval involvement. There
are two main forms of vulval LP: the papulosquamous variety,
which usually presents with itch, and the erosive variety, in
which tenderness, burning pain, dysuria and dyspareunia are the
usual presenting features.
Papulosquamous vulval lesions resemble the classical flattopped, purple papules with white streaks (Wickhams striae)
seen in cutaneous disease (Figure 4). Erosive LP tends to affect the
vaginal introitus and lower vaginal mucosa, sometimes with
fusion or loss of the labia minora. The eroded areas of skin may
bleed and produce copious exudate. Adhesions and severe vaginal
introital narrowing may develop in long-standing erosive LP.
Histological confirmation of a suspected diagnosis of lichen
planus is important as the dermatitis can present with features
suggestive of lichen sclerosus or infection. The development of

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Other inflammatory dermatoses


Dermatitis/eczema
Dermatitis or eczema can be endogenous (atopic or seborrhoeic
eczema) or endogenous (contact dermatitis or lichen simplex)
(Table 2).
Atopic dermatitis: atopic dermatitis of the vulva would be very
unusual without the more common manifestations of atopic
dermatitis elsewhere on the body and often a personal or family
history of dry skin, hay fever or asthma. Development of eczema
at this site may be precipitated by contact or friction factors or by
increased used of detergents to wash.
Contact dermatitis: contact dermatitis may be irritant or allergic.
The use of de-greasing agents such as soaps, bubble baths, feminine wipes etc., can all produce dryness of the skin with reduced

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Dermatitis in the vulval area


Type of dermatitis
Atopic dermatitis

Contact dermatitis

Seborrhoeic
dermatitis

Chronic lichenified
dermatitis
(Lichen simplex)

Features
Usually associated with typical atopic history
and evidence of atopic dermatitis elsewhere
on the body, such as flexural sites.
May be caused by allergens in direct contact
with skin of vulva or by allergens more
commonly touched by hands. See Table 3 for
possible culprits.
Glazed erythema, more uncomfortable than
itchy. May be evidence of pityriasis capitis
(dandruff ), facial seborrhoeic dermatitis or
similar inflammation in other flexural sites
such as axillae and sub-mammary.
Thickened, leathery skin, with some flaking.
Most common on labia majora and in crural
folds.

Table 2

barrier function and a vicious circle of itch, scratch and further


damage to the barrier of the skin. The skin will appear slightly dry
and flaky with mild inflammation.
Allergic contact dermatitis: allergic contact dermatitis can be
caused by a number of things which can come into contact with
vulval skin. Possible culprits are listed in Table 3. Patch testing to
elicit a type 4 hypersensitivity reaction can confirm the diagnosis
and avoidance of the allergen should solve the problem. The skin
will have become inflamed and more susceptible to irritant
dermatitis, so treatment with an anti-inflammatory topical steroid
plus emollient preparation will help the condition to settle fast.

Figure 5 Lichen simplex.

Seborrhoeic dermatitis, intertrigo and psoriasis: the three


conditions of seborrhoeic dermatitis, intertrigo and psoriasis can
present with identical features. The flexural areas of the vulva
(groin and interlabial sulci) are usually most severely affected with
glazed erythema and sometimes fissures or macerated skin. All may
affect other areas of skin so it is prudent to check for scaling in the
scalp (psoriasis and seborrhoeic dermatitis), nail irregularities
(psoriasis), flaking on the face in the nasolabial folds and eyebrows
(seborrhoeic dermatitis) or involvement of other flexural sites
(all three conditions). Swabs should be taken to check for candida
as well as bacterial infection and a skin biopsy can help to show
features of psoriasis, although seborrhoeic dermatitis and intertrigo
may have similar features.

Lichen simplex: lichen simplex, or chronic hyperkeratotic eczema,


can develop if the habit of scratching becomes established after the
development of a period of itch. Regular scratching or rubbing in
a localized area results in a leathery thickening of the skin with lowgrade inflammation, and scaling, sometimes plus erosions and
fissuring (Figure 5). The affected skin can resemble psoriasis,
hyperkeratotic LS or VIN.

Common contact allergens in vulval dermatitis


Type of allergen
Medicament
ingredients

Preservatives &
stabilizers
in creams
Perfumes
Clothing dyes
Metals

Urticaria
Although urticaria is usually a widespread skin disease, occasionally it affects limited areas of the body, especially sites where
skin meets mucosa. Itch may be a feature, but more often the
symptoms are a burning discomfort with swelling that lasts no
more than a few hours. Intercourse can trigger the episodes. Oral
antihistamine treatment will usually alleviate the condition.

Examples
Local anaesthetics, e.g. benzocaine,
dibucaine
Topical steroids, e.g. hydrocortisone
Neomycin, other antibiotics
Imidazoles, e.g. clotrimazole
Parabens, ethylenediamine, clioquinol

Plasma cell vulvitis


Histology of skin is necessary to make a diagnosis of plasma cell
vulvitis (vulvitis circumscripta plasmacellularis). It is a rare
condition of unknown aetiology, which causes slightly atrophic,
glistening bright red or yellow/orange plaques or patches limited
to the genital mucous membranes (Figure 6). The appearances
can mimic psoriasis or VIN but a skin biopsy will show a heavy
plasma cell infiltrate in the dermis. The eruption may be

Balsam of Peru, other fragrances


Paraphenylene diamine
Nickel

Table 3

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Figure 6 Plasma cell vulvitis.

asymptomatic, but some patients experience itching, burning and


tenderness. The abnormalities tend to resolve slowly after
treatment with topical corticosteroid, leaving post-inflammatory
hyperpigmention. Recurrences are common and some cases are
very recalcitrant, even suggesting treatment by surgical excision.
Blisters & ulceration
Ulceration can arise in hidradenitis suppuritiva or Crohns disease
as well as VIN or vulval cancer, but acute ulceration without preexisting inflammation suggests a number of other diagnoses.
Figure 7 Major aphthous ulcers.

Aphthous ulcers: small aphthous ulcers within the mouth are


common but can also affect the central vulval skin in a recurrent
manner. Occasionally larger ulcers develop, termed major aphthous ulcers, which can be up to 2 cm in diameter (Figure 7).
Although alarming and painful, these ulcers usually heal within
a week or two with conservative measures. The cause of aphthous
ulcers is not known, but they can be associated with iron or vitamin
B12 deficiency, streptococcal infection and inflammatory bowel
disease.

Behcets disease: the diagnosis of Behcets syndrome depends on


the fulfilment of certain criteria: recurrent oral ulceration plus two
of the following e recurrent genital ulceration; eye lesions (uveitis
or retinal vasculitis); skin lesions (erythema nodosum or acneiform nodules in adult); or a positive pathergy test. Thalidomide,
colchicine, dapsone, corticosteroids and other immunosuppressive agents have all been reported to help but no treatment is
universally effective and all carry the risk of serious side effects.

Acute vulval ulcer: in young women, the acute development of


a large ulcer could also be defined as an acute vulval ulcer (ulcus
vulvae acutum) or Lipschutz ulcer, which has been described as
a distinct entity. It has been associated with acute EBV and CMV
infection.

Pemphigus: pemphigus is a rare autoimmune blistering disease,


which affects the skin and mucous membranes with an onset
most commonly between the fourth and sixth decades. Vulval
involvement is not uncommon, especially if the disease is severe,
and occurs in approximately 10% of female cases.
Circulating antibodies against desmoglein protein in desmosomes, which are important for intercellular adhesion of
epidermal keratinocytes, leads to separation of skin cells and
intraepidermal blistering. Biopsy of perilesional skin with immunofluoresence is essential for diagnosis. Circulating antibodies
against epidermal adhesions will also be present, but can sometimes be found in other conditions where there are mucosal
erosions. Vulval involvement is more common than vaginal
lesions and usually present with pain, burning, erosion and
ulceration. Blisters are very fragile and frequently not seen.
Chronic disease can result in vaginal stenosis and vulval scarring.
Treatment involves immunosupression, initially in the form of
systemic corticosteroids, with the addition of steroid-sparing
agents such as azathioprine, ciclosporin, tacrolimus or mycophenolate mofetil once disease control is established. Patients

Nicorandil-induced ulcer: in an older woman, the development


of a painful ulcer with an undermined edge should always
prompt consideration of cancer, but must also include a nicorandil-induced ulcer. This entity has been described only recently
and is important to exclude. It can develop weeks to months after
starting nicorandil treatment, is painful but on stopping treatment, the pain often settles quickly although it can take some
weeks for the ulcer to heal.
Pyoderma gangrenosum: another diagnosis to consider in acute
ulceration is pyoderma gangrenosum. Associations include
inflammatory bowel disease, rheumatoid arthritis and haematological malignancies.
The biopsy may be non-specific, but in pyoderma gangrenosum
will show a mainly neutrophilic infiltrate at the ulcer edge.

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Neill SM, Tatnall FM, Cox NH. British Association of Dermatologists


guidelines for the management of lichen sclerosus 2010. Br J Dermatol
2010; 163: 672e82.

may need to remain immunosuppressed long-term to maintain


disease control.
A

FURTHER READING
BASHH. United Kingdom national guideline on the management of
anogenital warts. British Association for Sexual Health and HIV, www.
bashh.org; 2007.
Chan S, Harris M, Baldwin P, Sterling J. Vulvovaginal ulceration
during prolonged treatment with nicorandil. BJOG 2009; 116:
1403e5.
Criteria for diagnosis of Behcets disease. International study group for
Behcets disease. Lancet 1990; 335: 1078e80.
Jones RW, Scurry J, Neill S, MacLean AB. Guidelines for the follow-up of
women with vulval lichen sclerosus in specialist clinics. Am J Obstet
Gynecol 2008; 198: 496. e1e3.

OBSTETRICS, GYNAECOLOGY AND REPRODUCTIVE MEDICINE 21:6

Practice points
C

175

Always take a vaginal swab if there are symptoms of vaginal or


vulval itch or soreness. Candida or other infections are
common, either alone or in combination with other local
disease.
Consider allergic or irritant contact dermatitis from the use of
topical treatments.
If pre-malignancy cannot be excluded, tissue diagnosis is
essential.

2011 Published by Elsevier Ltd.

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