Case 13 A 68-year-old woman presents with long-standing vulval itch

 Case 13 A 68-year-old woman presents with

long-standing vulval itch

Edith Catchpole is 68-year-old retired secretary. For the last

8 years, she has suffered from vulva itch. Sometimes the itch

improves but it always flares up again. She cannot identify

anything that triggers this. She has tried a number of

different creams that she has bought from her local chemist.

These are mostly thrush treatments and creams for ‘personal

itch’. She has been too embarrassed to see her GP.

However, she can no longer cope as she often wakes up at

night scratching, disrupting her sleep and making her skin

painful. She feels that nothing really helps.

What are the most likely causes of itch

for this patient?

Vulva itch, known as pruritus vulvae, is a common

symptom which may be localized to the vulva or be part

of a more generalized skin disorder. You need to consider

likely causes in this age group with a long history and

fluctuating course of itch:

• Lichen sclerosus

• Lichen planus

• Lichen simplex

• Vulval intraepithelial neoplasia (VIN)

• Vulval thrush

What further questions would help to

establish the diagnosis?

You need to consider skin disorders and other relevant

factors so you need to ask questions beyond a standard

gynaecological history. You should ask about the

following.

1 Other skin conditions that she has or has experienced

in the past. The warm moist environment of the anogenital region means that the appearance may be atypical and

not obviously recognized. For example, psoriasis in the

vulva can appear as a well - demarcated, dark pink, moist

area without the typical silver scale.

2 Her personal or family history of atopic conditions as

well as any known allergies.

3 Her personal or family history of autoimmune disorders as some conditions (lichen sclerosus and lichen

planus) are associated with autoimmunity. Ask about

thyroid disorders, vitiligo, alopecia, diabetes and pernicious anaemia.

4 Any treatments she has tried so far (and their effects).

You should specifically ask if she is using any over - the -

counter preparations. She may even have tried treatments belonging to other family members or friends or

ones purchased abroad. Topical treatments may aggregate the underlying condition or result in an allergic

dermatitis.

5 Potential irritants including anything that comes in

contact with vulval skin such as clothes, washing products (for herself and her clothes) and sanitary products.

Mrs Catchpole tells you that she has been using 0.1%

hydrocortisone cream, prescribed by her GP, with some

improvement in her symptoms. She also used a cream that

she bought in a pharmacy in Spain which helped but she

finished this some time ago. She has been washing with

water only and using baby wipes to keep her vulva area

clean. She wears white cotton underwear and uses

blue-coloured toilet paper to match her bathroom suite. She

sometimes wears panty liners when she goes out as she has

occasional mild stress incontinence. She has an underactive

thyroid gland. She has taken thyroxine daily for the last 10

years with no problems. She is on no other medication. She

has no allergies and no other skin problems

Now review your list of possible causes

Lichen sclerosus

Lichen sclerosus can present at any age but is more commonly seen in postmenopausal women. It usually causes

severe itching and soreness and most commonly affects

Obstetrics and Gynaecology: Clinical Cases Uncovered.

By M. Cruickshank and A. Shetty. Published 2009 by Blackwell

Publishing. ISBN 978-1-4051-8671-1.Case 13 93

PART 2: CASES

the vulval skin but it can be found in other non - genital

sites. The whole vulva area and the skin around the anus

may be affected. Uncontrollable scratching may cause

bleeding and skin splitting and the skin becomes sore and

tender.

Lichen sclerosus is not linked with female hormone

changes, contraceptives, hormone replacement therapy

(HRT) or the menopause. It appears to be an autoimmune condition, with around 40% of women with lichen

sclerosus having or going on to develop another autoimmune condition.

Itch is related to active inflammation with erythema and

keratinization of the vulval skin. Hyperkeratosis can be

marked with thickened white skin. The skin can be fragile,

classically demonstrating subepithelial haemorrhages

(petechiae), and it may split easily. Typically, lichen sclerosus is seen around the introitus, perineum, labia minora,

clitoral region and perianal skin. Continuing inflammation results in inflammatory adhesions. Often, there is

lateral fusion of the labia minora which become adherent

and eventual disappear. The hood of the clitoris and its

lateral margins may fuse, burying the clitoris. Midline

fusion can produce skin bridges at the fourchette and narrowing of the introitus. Occasionally, the labia minora fuse

together medially which also restricts the vaginal opening

and can cause difficulty with micturition and even urinary

retention (Plate 13.1 ). Changes can vary in severity and

features may be defined but are often diffuse. Some areas

may be inflamed and red and others pale and crinkly or

thickened and white. Lichen sclerosus does not extend into

vaginal or anal mucosa.

Lichen planus

Lichen planus is a common skin condition that may

affect the skin anywhere on the body. Lichen planus

usually affects mucosal surfaces and is more commonly

seen in oral mucosa. Typically, lichen planus presents

with raised purplish plaques with a fine white reticular

pattern (Wickham ’ s striae). However, in the mouth and

genital region it can be erosive and more commonly associated with pain rather than itch. Erosive lichen planus

appears as well - demarcated glazed erythema around the

introitus. The aetiology is unknown but it may be an

autoimmune condition. It can affect all ages and does not

seem linked to hormonal status.

Lichen simplex

Women with sensitive skin, dermatitis or eczema can

present with vulva symptoms and these can result in

lichen simplex, a common inflammatory skin disease. It

presents with severe itch, especially at night, and is caused

by prolonged inflammation and scratching which thickens the skin. This is usually triggered by a chemical dermatitis but is sometimes linked to stress or low body iron

stores.

Specific allergic reactions can be found in the vulva

area from common allergens including perfumes, preservatives in topical treatments, rubber and textile dyes.

Washing powder, fabric conditioners, sanitary towels or

panty liners and synthetic underwear may be irritants.

Most women with a vulva disorder will benefit from

advice on general care of vulval skin and avoiding potential irritants (Box 13.1 ).

Vulva intraepithelial neoplasia

VIN is an uncommon premalignant condition of the

vulva skin with a similar natural history to cervical

intraepithelial neoplasia (CIN). Six per cent of women

with CIN (see Case 10 ) will also have VIN. However, not

all VIN is human papillomavirus (HPV) related, particularly in older women. HPV - related VIN is more common

in younger women. Without treatment, approximately

40 – 60% of cases will progress to cancer. The itch is

Box 13.1 Useful measures for vulval itch

Measures to improve vulval discomfort and irritation:

• Use a soap substitute to clean the vulval area. Water on

its own tends to cause dry skin

• Shower rather than bath and clean the vulval area only

once a day. Overcleaning can aggravate vulval

symptoms

• Wear loose fitting silk or cotton underwear

• Sleep without underwear

• Avoid fabric conditioners and biological washing

powders

• Avoid soaps, shower gel, scrubs, bubble baths,

deodorants, baby wipes or douches in the vulval area

• Some over-the-counter creams including baby or nappy

creams, herbal creams (e.g. tea tree oil or aloe vera) and

‘thrush’ treatments may include irritants

• Avoid wearing panty liners or sanitary towels on a

regular basis

• Avoid antiseptic (as a cream or added to bath water) in

the vulval area

• Wear white or light coloured underwear. Dark textile

dyes (black, navy) can irritate sensitive skin

• Use white toilet paper94 Part 2: Cases

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intractable although the use of emollients may help. It

appears as raised papules or plaques which can look

warty. It may be erythematous, white/keratotic or pigmented. Common sites are periclitoral, labia minora and

perineum. It can extend to perianal skin and it may also

be associated with vaginal intraepithelial neoplasia.

Vulval thrush

Vaginal thrush, characterized by a thick white discharge,

is more common than vulval thrush. With an anogenital

infection, irritation and soreness of the vulva and anus

are more likely and it is not associated with chronic itch

in postmenopausal women. Diabetes, obesity and antibiotic use may be associated. Vulval candidiasis may

become chronic and is more likely to present like intertrigo with a leading edge of inflammation extending out

from the labia majora to the inner thighs. Prolonged

topical antifungal therapy may be necessary to clear a

skin infection and advice on general care of the vulva

may reduce the predisposition to this condition.

Do you need to ask any more

specific questions?

Women may not volunteer information about sexual

function but this can be a distressing aspect of vulva

disorders.

On questioning, Mrs Catchpole says that she lives with her

79-year-old husband. They have not had penetrative sex for

the last 2 years as she is too sore and attempts at sex

resulted in skin splitting at the entrance to her vagina with

pain and some bleeding. She tells you that her husband is

very patient and although she is not particularly interested,

she misses the intimacy and knows that her husband would

like to resume a sexual relationship.

Genital examination

Many causes of vulval itch can be identified by examination of the external genitalia. It is important to examine

the vulva systematically with adequate light and exposure. This is best using a good light source with the

patient on an examination couch with legs raised into a

modified lithotomy position. Colposcopy is not necessary. You need to check labia majora, both sides of the

labia minora and clitoral hood, the fourchette, perineum,

perianal skin and natal cleft. It can be useful to use two

cotton buds to move skin folds to ensure complete

inspection.

If there is no obvious abnormality, you should ask the

patient to identify the symptomatic area. However, in

lithotomy position, a few women struggle to identify

the area themselves and some women may never self -

examine or be familiar with their vulva anatomy.

Speculum examination

There is no indication to perform a speculum examination as she has no vaginal symptoms. If she had features

of VIN, it would be important to examine other lower

genital tract sites including the vagina and cervix.

What further investigations might help

to distinguish the possible diagnoses?

You may want to confirm the diagnosis by biopsy. This

is not necessary unless you suspect VIN which must

be diagnosed histologically and invasion needs to be

excluded. You may reconsider biopsying if the diagnosis

is not obvious or at a later date if the patient does not

respond to treatment.

Let us review the examination findings

On examination, there is no evidence of skin disease at

non - genital sites. There are obvious areas of erythema

and pale crinkly skin which extends to the perianal area.

Both labia minora appear to be absent and the clitoris

not clearly defined. There are some telangectasia and a

small area of ulceration (Plate 13.2 ).

Do you need to take a biopsy?

The history and examination findings suggest a diagnosis

of lichen sclerosus. However, if you are suspicious of

the area of ulceration, you may want to take a biopsy

(Box 13.2 ).

KEY POINT

Vulva conditions often impact on sexual function and you

may need to ask specifically, especially in older women.

What would you look for

on examination?

General examination

Vulva symptoms may be the presenting feature of generalized skin disorders. You should look at common sites

for eczema, psoriasis, dermatitis and intertrigo including

hands, flexor and extensor aspects of joints, hairline

including behind ears, submammary and waist areas.Case 13 95

PART 2: CASES

The skin biopsy shows epidermal thinning, hydropic

degeneration of basal cells, hyalinization of the dermis and

subdermal lymphocyte infiltrate. Inflammatory changes are

seen in all zones of the skin but in particular there is a band

of inflammation in the dermis. These features support the

clinical impression of lichen sclerosus.

Can you now make a diagnosis?

These findings confirm your history and your clinical

examination findings.

Management

The main treatment is a prolonged course of ultra - potent

steroid cream. This is applied once daily to the whole

affected area for a month and then gradually reduced.

With lichen sclerosus, topical steroids should reduce the

itching quite quickly and any skin splits should begin to

heal. Loss of normal vulval architecture will not alter and

skin colour changes may not return to normal. Lichen

sclerosus is a lifelong condition but treatment and lifestyle changes can control symptoms very well.

An important part of the treatment is general care of

the vulval skin and avoidance of any potential irritants

that may worsen vulval irritation (Box 13.1 ).

Follow-up

You see Edith in 4 months’ time for review. She has

followed the reducing regime for the topical steroid cream

and is now using it once a week. She is delighted that her

itch and irritation have completely resolved. She continues to

use an emollient daily and soap substitute to wash in the

shower. However, she says that the skin still feels tight and

she is still unable to have sex.

On examination, the condition of her vulva skin is very

good with resolution of the erythema and hyperkeratosis.

However, the introital narrowing and reabsorption of her

labia minora remain unchanged.

Can you do anything to resolve

her aparunia?

Surgery may be offered if the lichen sclerosus has narrowed the vaginal opening so that it obstructs micturition or penetrative sex. This may require a Z - plasty or

local skin flaps to release scarring. Surgery itself will not

cure lichen sclerosus and it is important to have any

active inflammation controlled before surgery.

Edith decides that she does not want to undergo surgery

and will continue to use emollients and her steroid cream

when required to control any recurrence of her symptoms.

Does she require any further review?

There is a 4% lifetime risk of developing squamous cell

cancer of the vulva in the skin affected by lichen sclerosus. Edith needs to have information on this risk and to

know to report any ulcers or lumps that do not respond

to topical steroids.

Box 13.2 Vulval skin biopsy

Small diagnostic biopsies can be taken in the clinic under

local anaesthetic. A Keyes punch (Fig. 13.1) will produce

an adequate sample and avoiding crushing the tissue. You

can control any bleeding by pressure, silver nitrate pencil

or a undyed absorbable suture

Figure 13.1 A Keyes punch.

KEY POINT

Remember to discuss side-effects from steroids. Although

this can be a problem with oral steroids, skin thinning is

uncommon if a reducing regime is used to avoid excessive

use.96 Part 2: Cases

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CASE REVIEW

Vulval itch is a very common complaint and most women

will initially self - medicate. It is often self - limiting but

chronic vulval itch suggests an underlying vulval dermatosis or dermatitis secondary to use of potential irritants or

overcleaning the vulva area. History - taking and examination of the skin are fundamental to making a diagnosis.

Swabs should be taken if you suspect vulval thrush or a

superadded infection. Biopsies are not always necessary

unless you suspect VIN or invasive disease or if the condition does not respond to treatment. General care of vulva

skin and avoidance of potential irritants benefits most

conditions.

Lichen sclerosus is a chronic inflammatory skin which

is often seen in postmenopausal women. The incidence

appears to be in the order of 1 in 300 – 1000 of female adults

and children. There may be an autoimmune basis to the

development of lichen sclerosus. Over 20% of affected

women have one or more first degree relatives with autoimmune - related disorders. Alopecia and vitiligo are the

most commonly associated disorders but thyroid disease,

pernicious anaemia and diabetes mellitus are also seen.

The main symptom is severe itch but it also causes discomfort, pain and dysparunia. There is often distortion or

loss of the normal vulval architecture with labia minora or

clitoral fusion and introital narrowing. The skin can easily

split or tear. Many women find the symptoms embarrassing

and distressing and have concerns about sexual function.

The typical clinical appearance is pale atrophic wrinkled

skin, leucoplakia, telangectasia and erosions which form a

figure of eight distribution around the vulval and anal

areas. The symptoms often follow a fluctuating course with

episodes of reactivation.

The main aim of therapy is to achieve good symptom

control with the use of ultra - potent topical steroids and

general care of the vulva. However, anatomical damage

cannot be reversed. Surgery is limited to relieving symptoms secondary to labia fusion or introital narrowing. The

risk of squamous cell carcinoma is about 4%.

KEY POINTS

• Vulval itch is a very common complaint

• The history required for vulval skin disorders differs from a

standard gynaecological history as you need to know

about other skin disorders and related factors

• You need to ensure good positioning of the patient and a

good light source and take a systematic approach to

examining the anogenital region

• Skin biopsy is not always necessary unless you suspect

malignant disease or the condition does not improve in

response to first line therapy

• Advice on general care of vulval skin and avoiding irritants

often benefits women with vulval skin disorders in

addition to specific therapies

• Lichen sclerosus is a life-long condition but good

symptom control and prevention of tissue destruction can

be achieved with ultra-potent topical steroids

• Lichen sclerosus is associated with a 4% risk of squamous

cell cancer of the vulva and women need to know the

signs or symptoms to look out for

• Surgery is rarely required for lichen sclerosus except to

correct the effects of scarring such as aparunia or urinary

retention