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REVIEW
Skin diseases affecting
the vulva
Recurrence is common, particularly as Candida is a vulval
commensal. Poorly controlled diabetes, pregnancy and oral antibiotics all increase levels of Candida in the vulva, predisposing
to further infection. In difficult to treat cases, prophylactic fluconazole (150 mg weekly or monthly) may be a useful adjunct.
Shaheen Haque Hussain
Jane Sterling
Tinea cruris (groin ringworm): often occurs in conjunction
with tinea pedis (Athlete’s foot). In the groin, buttocks and upper
thighs, fungal infection causes well-demarcated erythematous
plaques with fine scaling, particularly at the leading edge. In hair
bearing areas, there may be deeper fungal involvement with
pustules or nodules. Skin scrapings of scaly areas for mycology
are useful to identify the causative fungus, generally Trichophyton species. If there is intercurrent tinea pedis, oral antifungal treatment with terbinafine or pulsed itraconazole to
treat the nail infection is necessary to prevent recurrence of the
groin rash.
Abstract
The vulva stretches between the mons pubis and the anus and is
bounded by the genitocrural folds. The epithelium includes the keratinised, 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.
Bacterial infections
Erythrasma: is caused by Corynebacteria, and may be confused
with tinea cruris. It presents with a faint, brownish discolouration of the groin, with fine peeling. Examination under a
Wood’s light (low wavelength ultraviolet light) demonstrates a
characteristic coral-pink fluorescence of affected skin. Erythrasma is more common in the immunosuppressed or diabetic
population. Treatment is with a topical antibiotic, such as
fusidic acid.
Keywords blister; infection; inflammation; pruritus vulvae; skin; ulcer;
vulva; vulvar diseases; vulvar neoplasms
Skin diseases affecting the vulva can cause significant morbidity
and impact negatively on quality of life. Patients are often
embarrassed by their symptoms, and may delay seeking a medical opinion.
In addition to a thorough history and examination, further
investigations including vulval swabs, skin biopsy or allergy
patch testing may be indicated to help reach the diagnosis
(Table 1).
Folliculitis: is visible as small papules and pustules associated
with hair follicles. A swab taken from pus within a pustule often
yields Staphylococcus aureus, but other aerobes and anaerobes
can also cause this infection. Diabetes, poor hygiene, immunodeficiency and obesity are all aggravating factors. Damage to the
skin through shaving presents with a similar picture, but the
infection tends to be superficial, without deeper involvement of
the hair follicles, and is termed pseudofolliculitis. Folliculitis
usually responds to topical antiseptics and systemic antibiotics,
although recurrence is common unless the course of treatment is
prolonged. General measures to reduce surface bacteria by
washing with antiseptic preparations 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 minimise
episodes of recurrence. In recurrent/difficult to manage cases, it
is worth taking a nasal swab to identify chronic Staphylococcal
carriers. Appropriate eradication treatment may help in these
cases.
Occasionally infection from an infected follicle can burst into
the adjacent subcutaneous tissue forming a larger inflammatory
mass with a collection of pus recognisable as a boil or furuncle.
Infections
Given the relative warmth and moisture of the vulva, infections
in this area are common. It is important to remember that superadded infection can complicate and exacerbate a pre-existing
inflammatory dermatosis, making treatment of the underlying
condition more difficult.
Yeast & fungal infections
Vulvovaginal candidiasis: is characterised by itch and usually a
thick, creamy discharge. A vaginal swab should be taken to
confirm the diagnosis before initiating treatment with topical
imidazoles (in a pessary or intravaginal cream formulation), or
orally. Chronic or incompletely treated candidiasis can present
with persistent vulval erythema and fissuring in the interlabial
sulci or perineum. Recurrent episodes may respond best to a 7
e10 day course of oral fluconazole. Episodes may be precipitated
by antibiotic use or associated with the menstrual cycle.
Vulval cellulitis: 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 from a vulval swab may confirm the organism (usually
Streptococcus pyogenes) but treatment should be started immediately to reduce the risk of secondary lymphatic damage and the
possibility of lymphoedema with recurrent episodes. Penicillin,
flucloxacillin or erythromycin are appropriate choices for oral
antibiotics.
Shaheen Haque Hussain MB BS BSc MRCP is a Consultant in the Department of Dermatology at Addenbrooke’s Hospital, Cambridge, UK.
Conflicts of interest: none declared.
Jane Sterling MB BChir MA FRCP PhD FHEA is a Consultant in the Department
of Dermatology at Addenbrooke’s Hospital, Cambridge, UK. Conflicts of
interest: none declared.
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Ó 2014 Elsevier Ltd. All rights reserved.
Please cite this article in press as: Haque Hussain S, Sterling J, Skin diseases affecting the vulva, Obstetrics, Gynaecology and Reproductive
Medicine (2014), http://dx.doi.org/10.1016/j.ogrm.2014.04.003
REVIEW
Anogenital warts: in adults are generally sexually transmitted,
and are usually caused by low-risk human papillomaviruses
(HPV) 6 or 11. They present as small papules or cauliflowershaped growths, and are most commonly found at the posterior fourchette, but can occur at any site within the vulva or
vagina.
Treatment of vulval warts depends on the number, size and
location. Topical antiproliferative treatments (podophyllotoxin,
trichloroacetic acid) or destructive techniques (cryotherapy) are
most commonly used. Immunotherapy with topical imiquimod is
also effective, and may reduce recurrence rates. Treatment is
more difficult in the immunosuppressed, as an immune response
against the virus is necessary to clear the infection. Keratinised
warts may not respond as effectively to treatment due to poor
penetration, and a surgical approach with curettage or shave
excision may be more successful.
It is important to undertake a cervical cytology sample in
these patients to exclude HPV-associated cervical disease.
Presentation of vulval disease
Symptom
Possible diagnosis
Acute presentation Chronic continuous/intermittent
Itch
Candida
Lichen simplex
Contact dermatitis Lichen sclerosus
Lichen planus
Tinea cruris (ringworm)
Partially controlled contact
dermatitis
Incompletely treated candida
Discomfort/ Irritant dermatitis Psoriasis
Pain
Folliculitis
Seborrhoeic dermatitis
Cellulitis
Intertrigo
Boils
Erythrasma
Lichen sclerosus
Lichen planus
Plasma cell vulvitis
Vulvodynia/Vestibulodynia
Swelling of Cellulitis
Urticaria
vulva
Hidradenitis suppuritiva
Crohn’s disease
Lymphoedema (primary or
secondary)
Lump(s)
Warts
Cysts
Molluscum
Hidradenitis suppuritiva
contagiosum
Malignancy
Blisters
Herpes simplex
Pemphigus (vulgaris or foliaceous)
Ulcers
Aphthous ulcers
Nicorandil-induced ulceration
Aphthous ulcers
Behc‚et’s disease
Disorders associated with malignancy
Vulval intraepithelial neoplasia (VIN): is commonly associated
with HPV 16, and has a varied clinical presentation. Symptoms
include irritation, mild discomfort, pain or ulceration. It can
however be asymptomatic, and a change in texture and/or
appearance is noted. On examination, VIN presents as an area of
glazed erythema, thickened, macerated skin, an area of brown
pigmentation or a combination of these features (Figure 1). VIN
patients are more likely to develop cervical intraepithelial
neoplasia (CIN) and anal intraepithelial neoplasia (AIN), and
should be examined to exclude this.
There are two main histological subtypes of VIN e well
differentiated and basaloid. The latter is more common, particularly in pre-menopausal women. Untreated VIN may progress to
invasive squamous cell carcinoma (SCC) in approximately 5% of
cases. Unifocal disease may be best treated surgically, and
recurrence is unlikely if clear margins are obtained. Multifocal or
recurrent disease poses a greater problem as extensive vulval
surgery may be mutilating. Alternative treatments include laser
and topical immunotherapy with imiquimod or cidofovir. Both
topical treatments have been reported to clear VIN, but treatment
produces an intense inflammatory reaction with discomfort and
occasionally ulceration which limit their efficacy.
Table 1
Viral infections
Genital herpes simplex virus: (HSV) infection is not uncommon and is generally diagnosed in primary care or genitourinary clinics. Infection is usually with HSV2, but rates of genital
HSV1 infection are increasing. Primary genital HSV in a previously seronegative individual causes symptoms within 7 days
of infection. There may be a prodromal phase with localised
pain prior to the development of papules and vesicles which
ulcerate and are extremely painful. There can 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. Oral aciclovir will
help to shorten the duration of the acute eruption provided it is
taken early in the course of the infection (within 5 days of
symptom onset if new lesions are forming). If episodes are
frequent, regular prophylactic acyclovir (200 mg twice daily for
6 months) can help to reduce the number of reactivation
episodes.
Lichen sclerosus (LS): most frequently presents with itch, but is
often initially misdiagnosed as Candida. Soreness and dyspareunia are also common complaints, but occasionally LS can be
asymptomatic. Examination reveals white, shiny patches classically in a ‘figure of eight’ distribution affecting the vulva, perineum and perianal skin (Figure 2). The clitoral hood, clitoris,
labia minora and interlabial sulci are also commonly affected.
Other skin changes include bruising (ecchymoses), telangiectasia, thickening, hyperkeratosis, fissuring and ulceration. The
vaginal mucosa is not involved but loss of elasticity and sclerotic
change at the vaginal introitus can make intercourse painful.
Patients with long-standing LS may develop architectural change
due to scarring secondary to chronic inflammation damaging to
the dermis and dermo-epidermal junction. This may be evident
as burying of the clitoris, resorption of the labia minora, anterior
Molluscum contagiosum: produces small pearly papules with a
central shallow pit 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.
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Please cite this article in press as: Haque Hussain S, Sterling J, Skin diseases affecting the vulva, Obstetrics, Gynaecology and Reproductive
Medicine (2014), http://dx.doi.org/10.1016/j.ogrm.2014.04.003
REVIEW
Figure 1 VIN-vulval intraepithelial neoplasia.
Figure 2 Lichen sclerosus.
mammary Paget’s is related to an underlying gastrointestinal,
urological or gynaecological adenocarcinoma; so thorough
investigation is required in patients who present with this
condition.
The affected skin is usually sore, thickened and macerated
with an eroded surface (Figure 3). Histology shows mucincontaining cells within the epidermis and a variable degree of
surface erosion and dermal inflammatory cell infiltrate.
Treatment of vulval Paget’s disease is difficult as clinically
uninvolved skin may show histological features of Paget’s, thus a
surgical approach to achieve clearance of the disease may be
extensive. Laser ablation of the skin can help symptomatically
but does not treat deeply enough to eradicate disease in follicular
epidermis, so recurrence rates are higher. Imiquimod can also be
used. In many cases, the disease remains static so a symptomatic
approach to treatment can be considered plus regular review and
observation.
or posterior fourchette fusion and occasionally complete fusion
of the labia minora with loss of the introitus causing difficulty
with micturition or even retention.
LS is associated with autoimmune 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 (which generally resolves following puberty). The
lifetime risk of development of vulval squamous cell carcinoma
in adults is approximately 5%.
The inflammatory process can be controlled with an ultrapotent topical corticosteroid. Clobetasol proprionate 0.05% is
the mainstay of treatment in the UK, but other potent steroid
preparations may be sufficient. After regular daily use for 1
month, frequency of application can be reduced, but as the
inflammation is chronic, treatment may 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 (koebnerisation) but is essential if invasive
SCC develops and may be necessary if pre-malignancy is suspected or scarring/fusion impede micturition.
Lichen planus: (LP) 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: papulosquamous
(typically itchy) and erosive (usually presenting with tenderness,
burning, dysuria and dyspareunia).
Papulosquamous vulval lesions resemble the classical flattopped purple papules with white streaks (Wickham’s striae)
seen in cutaneous disease (Figure 4). Erosive LP tends to affect
Extra-mammary Paget’s disease: is classified as primary (not
associated with systemic disease) or secondary. Secondary extra-
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Please cite this article in press as: Haque Hussain S, Sterling J, Skin diseases affecting the vulva, Obstetrics, Gynaecology and Reproductive
Medicine (2014), http://dx.doi.org/10.1016/j.ogrm.2014.04.003
REVIEW
Figure 3 Extra-mammary Paget’s disease.
Figure 4 Lichen planus, papulosquamous form.
history of eczema, asthma or hay fever. It may be precipitated by
friction or irritation in a susceptible individual.
Contact dermatitis is either irritant or allergic in nature. Soaps,
bubble baths, feminine wipes etc can cause an irritant dermatitis
through degreasing and drying of the sensitive vulval skin,
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 LP is
important as the dermatitis can present with features suggestive
of LS or infection. The development of SCC is rare, but can occur,
especially with 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 pessaries, 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
koebnerisation in scar sites and the risk of developing postoperative synechiae.
Dermatitis in the vulval area
Type of dermatitis
Features
Atopic dermatitis
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.
Contact dermatitis
Seborrhoeic
dermatitis
Other inflammatory dermatoses
Dermatitis/eczema: encompasses both endogenous (atopic or
seborrhoeic) and exogenous (contact dermatitis or lichen simplex) forms (Table 2).
Atopic dermatitis affecting the vulva is normally accompanied
by evidence of eczema elsewhere, and a personal and/or family
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Chronic lichenified
dermatitis
(Lichen simplex)
Table 2
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REVIEW
thereby reducing its barrier function and setting up a vicious
circle of itch, scratch and further damage to the skin.
An allergic contact dermatitis is a type IV hypersensitivity
reaction caused by prior sensitisation to an allergen. Common
allergens are listed in Table 3. Patch testing to identify the
allergen confirms the diagnosis, and avoidance will ameliorate
symptoms. A secondary irritant dermatitis may co-exist, so a
topical steroid and emollient preparation will help symptoms
settle quickly.
Lichen simplex,: or chronic hyperkeratotic eczema, can develop
if the habit of scratching becomes established after a period of
persistent itch. Regular scratching or rubbing in a localised area
results in a leathery thickening of the skin with low-grade
inflammation, and scaling (Figure 5). Erosions and fissures can
also occur. The affected skin can resemble psoriasis, hyperkeratotic LS or VIN.
Psoriasis, seborrhoeic dermatitis and intertrigo: can present
identically with glazed erythema and fissures or macerated skin
affecting the vulval flexures. It is important to examine extensor
surfaces (elbows & knees), nails and scalp for other areas of
psoriasis; the scalp, flaking in the nasolabial folds and eyebrows
for seborrhoeic dermatitis and involvement of other flexural
areas for all three conditions. A swab should be taken to check
for co-existent Candida or other bacterial infection.
Urticaria: is generally a widespread skin disease, but can occasionally affect the vulval area locally where it presents with
swelling and a burning discomfort that lasts no more than a few
hours. Oral antihistamines are the treatment of choice.
Figure 5 Lichen simplex.
VIN, but a skin biopsy will show a heavy plasma cell infiltrate in
the dermis. It can be asymptomatic, or cause itching, burning or
tenderness. Treatment with a topical steroid can lead to slow
resolution with post-inflammatory hyperpigmentation. Recurrence is common, and recalcitrant cases may be treated with
surgical excision in extreme cases.
Plasma cell vulvitis: (vulvitis circumscripta plasmacellularis) is
a diagnosis made histologically. It is uncommon, of unknown
aetiology and 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
Blisters & ulcers
Common contact allergens in vulval dermatitis
Type of allergen
Aphthous ulcers: within the mouth are common. These are
typically small, and can also affect the vulval skin. Occasionally
Examples
Medicament ingredients Local anaesthetics, e.g. benzocaine,
dibucaine
Topical steroids, e.g. hydrocortisone
Neomycin, other antibiotics
Imidazoles, e.g. clotrimazole
Preservatives &
In creams:
stabilisers in creams
Parabens, ethylenediamine, clioquinol
In moist wipes:
Methylchloroisothiazolinone/
methylisothiazolinone (previously called
Kathon CG)
Perfumes
Balsam of Peru, other fragrances
Clothing dyes
Paraphenylenediamine
Metals
Nickel
Table 3
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Figure 6 Plasma cell vulvitis.
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Please cite this article in press as: Haque Hussain S, Sterling J, Skin diseases affecting the vulva, Obstetrics, Gynaecology and Reproductive
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REVIEW
found in desmosomes which are important in keratinocyte
adhesion) lead to separation of skin cells and blister formation. A
skin biopsy of the ulcer edge and perilesional skin for immunofluorescence is essential for diagnosis and shows an intraepidermal blister. Vulval involvement is more common than
vaginal lesions, and present with pain, burning, erosions and
ulceration. Intact blisters are often not seen. Chronic disease can
cause vaginal stenosis and vulval scarring. Treatment involves
immunosuppression, initially oral steroids and then steroidsparing agents including azathioprine, ciclosporin or mycophenolate mofetil when the disease is under control. Long-term
immunosuppression is often needed to maintain disease
remission.
Behcet’s disease: is a rare condition characterised by recurrent
oral ulceration and two of the following additional criteria:
recurrent genital ulceration, ocular involvement (uveitis or
retinal vasculitis), skin involvement (erythema nodosum or
acneiform nodules) or a positive pathergy test (sterile pustules at
the site of mild trauma). Thalidomide, colchicine, dapsone, corticosteroids and other immunosuppressants have all been reported to help, but no treatment is universally effective and all
carry the risk of serious side effects.
Nicorandil-induced ulceration: is a recently described entity.
Nicorandil is a vasodilator used to treat angina. Patients can
develop a painful ulcer with an undermined edge within weeks to
months after starting nicorandil. On stopping treatment, the pain
settles quickly, although the ulcer may take several weeks to heal.
Figure 7 Major aphthous ulcers.
Other conditions affecting the vulva
major aphthous ulcers (up to 2 cm in diameter) can develop
(Figure 7). These ulcers are acutely painful but heal within 1e2
weeks with conservative measures and analgesia. The cause of
aphthous ulceration is unknown, but associated factors include
nutritional deficiencies (iron or Vitamin B12), streptococcal
infection and inflammatory bowel disease.
Hidradenitis suppurativa (HS): is characterised by recurrent
boils and abscesses affecting flexural skin including the groin,
axillae and sub-mammary areas. The inflammatory process can
lead to sinus tracts and scarring. HS is associated with other
‘follicular occlusion’ conditions such as acne, and may be familial. Patients should be advised to lose weight and stop
smoking. Topical antiseptic washes and topical antibiotics are
useful for mild disease. Tetracycline antibiotics are the first-line
treatment, followed by a combination of rifampicin and clindamycin. Isotretinoin (an oral retinoid), dapsone and infliximab
can also be used.
} tz ulcer
Acute vulval ulcer: (ulcus vulvae acutum) or Lipschu
has been described as a separate entity to aphthous ulceration,
and may be associated with acute EBV or CMV infection.
Pyoderma gangrenosum: presents as a painful ulcer with a
violaceous, undermined edge. It is associated with inflammatory
bowel disease, rheumatoid arthritis and haematological disorders, but can also be idiopathic. Lesions can develop at any site
on the skin, but is often triggered by mild trauma. A skin biopsy
demonstrates a neutrophilic infiltrate at the ulcer edge, but may
be non-specific. Treatment is immunomodulatory with topical or
oral steroids initially.
Vulval Crohn’s disease: can affect up to 30% of patients with
Crohn’s disease, and has similar features to chronic cellulitis or
HS. It can pre-date, or occur in conjunction with intestinal disease. Patients present with marked swelling and oedema of the
vulval skin with pustules, ulcers, fissures, sinuses, fistulae and
induration. A skin biopsy can be non-specific, or may show noncaseating granulomatous inflammation. Treatment of the underlying inflammatory bowel condition with immunosuppression can help genital disease. Topical treatment with potent
corticosteroids can help superficial lesions.
A
Pemphigus: is a rare autoimmune blistering condition affecting
the skin and mucosal membranes with onset most commonly
between the fourth and sixth decades. Vulval involvement is not
uncommon, occurring in approximately 10% of cases. Patients
present with flaccid blisters, and the skin may demonstrate
Nikolsky’s sign (where gentle rubbing or pressing of the skin
causes blister formation due to the epidermis separating from the
dermis). Circulating antibodies against desmoglein (a protein
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FURTHER READING
BASHH. UK National guideline on the management of vulval conditions.
2007. Clinical Effectiveness Group, British Association for Sexual
Health and HIV, www.bashh.org.
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Please cite this article in press as: Haque Hussain S, Sterling J, Skin diseases affecting the vulva, Obstetrics, Gynaecology and Reproductive
Medicine (2014), http://dx.doi.org/10.1016/j.ogrm.2014.04.003
REVIEW
BSSVD. Standards of care for women with vulval conditions. 2013. British
Society for the Study of Vulval Disease, www.bssvd.org.
Chi C-C, Kirtschig G, Baldo M, Brackenbury F, Lewis F, Wojnarowska F.
Topical interventions for genital lichen sclerosus. Cochrane Database
Syst Rev 2011 Dec 7; http://dx.doi.org/10.1002/14651858.CD008240.
pub2. Issue 12. Art. No.: CD008240.
OBSTETRICS, GYNAECOLOGY AND REPRODUCTIVE MEDICINE --:-
Lynch PJ, Moyal-Barracco M, Scurry J, Stockdale C. 2011 ISSVD terminology and classification of vulvar dermatological disorders: an approach
to clinical diagnosis. J Low Genit Tract Dis 2012; 16: 339e44.
Neill SM, Tatnall FM, Cox NH. British Association of Dermatologists’
guidelines for the management of lichen sclerosus 2010. Br J Dermatol
2010; 163: 672e82.
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Please cite this article in press as: Haque Hussain S, Sterling J, Skin diseases affecting the vulva, Obstetrics, Gynaecology and Reproductive
Medicine (2014), http://dx.doi.org/10.1016/j.ogrm.2014.04.003