Pruritus vulvae and vulval pain are very common complaints and most women initially self medicate. Although it is often selflimiting, chronic vulval pruritus suggests an underlying vulval dermatosis.
Careful and systemic examination is fundamental to making a diagnosis.
Skin biopsies are not always necessary but are essential if VIN or invasive disease is suspected or if the condition does not respond to treatment.
General care of vulval skin is a fundamental component of treatment.Avoidance of potential irritants will benefit most conditions.
The mainstay of the management of lichen sclerosus is topical ultrapotent steroids. Women require clear advice on the appropriate treatment regimes.
Women with VIN require a biopsy to confirm disease.Longterm surveillance is necessary, particularly when a medical or conservative approach to management is taken.
All gynaecological trainees require experience in the management of common skin disorders, but a specialist service improves care for women by improving the accuracy of diagnosis and the implementation of adequate and appropriate treatment.
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Vulval skin disorders
1. Wafaa B. Basta
Specialist Gynaecology & Obstetrics at Mataria Teaching
Hospital
MBBch., MSc ., Egyptian Fellowship, MRCOG
ERC MEMBER
27th scientific meeting CPD Program –ERC RCOG
Soneasta 4th September 2011
2. Although the vulva is the most visible female
genital structure, it has received the least
attention in the medical literature and has
even been referred to as “the forgotten
pelvic organ”
Vulvar disorders entails a wide variety of
diseases each ,has a lot of D.D.,e.g.
1. Benign non-infectious skin disorders
(dermatosis)
2. Infectious vulval skin disorders (STD)
3. Pre-invasive & invasive cancer
3. Group of conditions characterized by
inflammation of the vulval skin, presenting
mostly with pruritis.
This group once refered to as” vulval
dystrophy “is now termed “benign non-
infectious vulval dermatosis “
It is also refered to as non-neoplastic
epithelial disorders.
4. Vulvar dermatoses may present in a variety
of ways, ranging from asymptomatic to
chronic disabling conditions, which are often
difficult to treat and severely impact a
woman’s quality of life.
All of these conditions may present with
pruritis. Other associated symptoms may
include pain, dyspareunia , fissuring, change
in colour & texture of skin and bleeding after
intercourse.
The management of women with chronic
benign vulvar skin disorders has been one of
the most difficult and challenging aspects of
women's healthcare for a long time.
5. There are many ways in which the keratinized
skin and muco-cutaneous surfaces of the vulva
differs from skin on the rest of the body:
1. it is the only area of the human body where
epithelium from all three embryologic layers
coalesce.
2. In addition, because the vulvo-vaginal tract
contains foreign proteins and antigens
necessary for reproduction, this area of the
body has a unique immunologic response .
3. Lastly, the subcutaneous tissue of the labia
majora is looser, allowing for considerable
oedema to form .
6. Communitybased surveys in UK indicate that
about onefifth of women have significant
vulval symptoms.
In the hospital setting, common causes are
dermatitis,lichen simplex,vulval candidiasis,
lichen sclerosus and lichen planus.
Lichen sclerosus accounts for at least 25% of
the women seen in dedicated vulval clinics.
8. To provide an evidencebased framework for
improving the initial assessment and care of
women with vulval disorders .
To describe the presentation and management
of the major,common vulvar dermatoses & its
D.D .
Be familiar with the new terminology for vulval
skin disorders.
Advice on general care of the vulval skin
Evaluate the need for specialized clinics,with
MDT including dermatologist,patch test services ,
psychosexual councellers & reconstructive
surgeons .
9. Dystrophy” is no longer an acceptable term; the
new ISSVD classification system lists specific
dermatologic disorders .
The Terminology Committee presented a new
classification of the benign, non-infectious
vulvar dermatoses to the ISSVD membership at
the February 2006 World Congress meeting.
The recent terminology has been published by
PJ Lynch, MD, M Moyal-Barrocco, MD, F
Bogliatto,MD, L Micheletti, MD and J Scurry, MD .
Lynch PJ, Moyal-Barrocco M, Bogliatto F,
Micheletti L, Scurry J. 2006 ISSVD classification
of vulvar dermatoses: pathologic subsets and
their clinical correlates. Journal of Reproductive
Medicine. 2007;52(1):3-9.
14. -Prevalence: 10-15% of population
-If 2 parents with eczema, 80% risk to
children
-Criteria for diagnosis
1-Itching/ scratch cycle
2-Exacerbations and remissions
3-Eczematoid lesions on vulva and
elsewhere(cruralfolds,scalp,
umbilicus, extremities)
4-Personal or family of hay fever,
asthma, rhinitis, or other allergies
5-Clinical course longer than 6 weeks
-Treatment:
1-Avoid scratching;
2- stress management
3-Emollients (bland, petrolatum
based)
4-Topical steroids (moderate
potency)
5-Intralesional triamcinolone
Tacrolimus (Protopic) 0.03% to
0.1% BID
6-Oral antihistamines or doxypin
5% cream
15. Allergic contact dermatitis Irritant contact dermatitis
Delayed hypersensitivity
10-14d after first
exposure; 1-7d after
repeat exposure
Itching, burning,
swelling, redness
Small vesicles or bullae
more likely with ACD
Elicited in most people
with a high enough dose of
irritant:
Potent irritant: chemical
burn
Weaker irritant: applied
repeatedly
Rapid onset vulvar itching
(hours-days)
Itching, burning,
swelling, redness
17. -Present with burning, itching,
dyspareunia, and fissuring around
the introitus .
-Examination : erythema , edema.
-Continued exposure may lead to
lichen simplex chronicus (LSC).
-The diagnosis is made by taking a
detailed history and careful physical
examination.
-One should have a low threshold to
perform a biopsy and rule out
coexisting conditions.
-The differential diagnosis includes
candidiasis, psoriasis, sebhorreic
dermatitis, LSC, and extensive extra
mammary Paget’s disease.
- Patch testing may be helpful in
making the diagnosis.
18. -The cornerstone of treatment is
identification and removal of the caus-
ative irritant or allergen.
-Topical steroids.
-Ice packs
-Antihistamines such as hydroxyzine .
- Low-dose tricyclic antidepressants
such as amitriptyline can be used to
help women stop scratching in their
sleep.
- Instruct patients in proper vulvar
hygiene.
-Topical steroids and tricyclic
antidepressants should be tapered
gradually .
- Superimposed candidal, bacterial
infections should be treated .
- For patients who report minimal or
no improvement, the diagnosis should
be re-evaluated .
19. -It can involve the skin of the
vulva but not vaginal mucosa.
-The appearance of vulval
psoriasis differs from the typical
scale of nongenital sites:it often
appears as smooth,nonscaly red or
pink discrete lesions.
- Scratching may cause infection,
dryness and skin thickening.
- Examination of other sites
including nails and scalp may be
helpful in making a diagnosis.
- Emollients, soap substitutes,
topical steroids and calcipotriene
are useful for symptom control ,
but cold tar preparations should
not be used in genital sites
20. -Also called chronic vulval dermatitis
-Either Primary (idiopathic) or Secondary
(superimposed on lichen sclerosus, lichen
planus, or other vulvar disease)
-A common inflammatory skin condition.
-Presents with severe intractable pruritus,
especially at night.
-Involves the labia majora but can extend to
the mons pubis and inner thighs.
-There may be erythema and swelling with
discrete areas of thickening and
lichenification,especially with scratching.
-Are sometimes linked to stress or low body
iron stores.
-The mainstay of treatment is general care of
the vulva , avoiding potential irritants and
use of emollients and soap substitutes.
-Antihistamines or antipruritics may be
helpful, especially if sleep is disturbed.
-However, moderate or ultrapotent topical
steroids may be necessary to break the itch–
scratch cycle
21. -It is a destructive inflammatory
condition
-Any age, but is more common in
postmenopausal women & in
children.
-Incidence:1in 300-1in 1000 in all
races ,increase in white women.
-Any body site with a predilection
for genital skin
-Not linked to hormone changes,
COC,HRT or the menopause.
-Evidence suggests that it is an
autoimmune condition,(40%)
-Genetic predisposition plays a role
-It causes severe pruritus, worse at
night.
-The whole vulval perianal area
may be affected in a figure ofeight
distribution.
-Uncontrollable scratching may
cause trauma with bleeding and
skin splitting and symptoms of
discomfort, pain & dyspareunia
22. -Hyperkeratosis can be marked with
thickened white skin.
-The skin is often atrophic, classically
demonstrating subepithelial
haemorrhages (ecchymoses),and it
may split easily.
-Continuing inflammation results in
inflammatory adhesions.
-Often there is lateral fusion of the
labia minora, which become adherent
and eventually are completely
reabsorbed.
-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
23. -Is a common skin disease
-May affect the skin anywhere
on the body.
-Usually affects mucosal
surfaces and is more commonly
seen in oral mucosa.
-The aetiology is unknown, but
it may be an autoimmune
condition.
- It can affect all ages and is not
linked to hormonal status
24. -Presents with polygonal flat-
topped violaceous purpuric
plaques and papules with a fine
white reticular pattern
(Wickham striae).
-However, in the mouth and
genital region it can be erosive
and is more commonly
associated with pain than with
pruritus.
- Erosive lichen planus appears
as a well demarcated, glazed
erythema around the introitus.
25.
26.
27. -Is a chronic inflammatory bowel
disorder.
- It can involve the vulva by direct
extension from involved bowel or
‘metastatic’ granulomas.
- Rarely, it is seen without known
bowel disease or preceding the
presentation of bowel disease.
-The vulva is often swollen and
odematous with granulomas,
abscesses, draining sinuses and
ulceration.
-Surgery can result in sinus and
fistula formation and tissue
breakdown and therefore should be
avoided.
-Treatments include metronidazole
and oral immunomodulators.
28. -Is a chronic multisystem
disease
-Characterised by recurrent
oral and genital ulcers.
-In women,ulcers can involve
the cervix,vulva or vagina.
-The ulcers are usually recurrent
and painful and can leave
scarring.
-Treatment to control flareups
and reduce symptoms is based
on topical or systemic immuno-
suppressants.
29. -Is a rare benign chronic
inflammatory condition of the
vulva
-Presents with pruritus
,burning, dyspareunia and
dysuria.
-Usually in postmenopausal
women.
-Is diagnosed histologically and
is characterised by dermal
infiltration with plasma cells,
vessel dilatation and
haemosiderrin deposition.
- The aetiology is unknown; one
theory is that it is an
autoimmune disorder.
-There have been case reports
favouring successful treatment
with topical ultra-potent
steroids
30. Vulvar skin disorders are often improperly diagnosed and
treated because of many reasons:
1. Patients embarrassment :make them reluctant to seek
medical advice early.
2. Self medication: as local medications may alter the gross
picture of the disease .
3. Superimposed bacterial &fungal infection: may be
misleading.
4. Discrepancy in nomenclature of different vulvar skin
disorders
5. Wide range of D.D.of vulval dermatomes, STD, VIN &
vulval cancer makes it a challenging situation for the
gynaecologist .
6. The gynaecologists lack of training & experience on
dermatologic disorders
7. The Dermatologists -who are most familiar with skin
diseases- are infrequently trained in vulvo-vaginal
examination.
31. These factors may result in women receiving
suboptimal treatment, resulting in persistent
symptoms and progression of the disease.
So, accurate diagnosis depends on carful
detailed history taking, meticulous
examination, investigations if needed &
keeping in mind all the diffrential diagnosis
with its characteristic features & prevalence.
32. Pruritus and pain are the most common
presenting symptoms(Nonspecific).
The nature , duration, periodicity of
symptoms, its aggravating &relieving factors .
Abnormal cervical cytology,(VIN)
Cigarette smoking and immune deficiency
(VIN).
Contact with potential allergens .(contact or
allergic dermatitis)
33. o Personal or family history of:
Atopic conditions (hay fever, asthma, ...).
Eczema, psoriasis
Autoimmune conditions. ( lichen sclerosus and erosive lichen
planus ).The most common autoimmune conditions in women
with lichen sclerosus are thyroid disorders,alopecia
areata,pernicious anaemia,type 1 diabetes and vitiligo
Symptoms of urinary or faecal incontinence. ( damages the
vulval skin either directly or indirectly by the use of sanitary
products).
Other sites involvment: mouth, eyes, elbows, scalp
Current& previous medications & response (Antibiotics,
hormones, steroids, etc)
Skin care: soaps, baby wipes, menstrual pads, new clothing,
scrubbing, etc (contact , allergic dermatitis)
New sexual partner(s); barrier contraceptives (allergy, STD)
34. Systematically examine the vulva with adequate
light and exposure. ( modified lithotomy position
with a good light source).
Colposcopy is not necessary in every case.
Ask the woman to identify the symptomatic
area.
If VIN is suspected, examine other lower genital
tract sites including the vagina, cervix and peri-
anal skin.
Examine the rest of the body, including the
mouth, for signs of lichen planus and the scalp,
elbows, knees and nails for psoriasis. Eczema
may be seen at any site.
35. In the initial assessment of a woman with vulval symptoms, consider testing for
thyroid disease, diabetes and sexually transmitted infections if clinically
indicated. { D }
Skin biopsy is not necessary when a diagnosis can be made on clinical
examination. Biopsy is required if the woman fails to respond to treatment or
there is clinical suspicion of VIN or cancer. { D }
VIN is a histological diagnosis and a biopsy must be taken. On excision, 19–22% of
cases of VIN have unrecognised invasion detected.
Women suspected of having lichen sclerosus or lichen planus should be
investigated for other autoimmune conditions if there are clinical symptoms or
signs. { C }
No evidence has been identified to support testing for autoantibodies without a
clinical indication.
Serum ferritin should be checked in women with vulval dermatitis. {C}
Correction of irondeficiency anaemia or low serum ferritin can relieve vulval
symptoms.
o Skin patch testing should be performed for women seen with vulval dermatitis.
{ D } 26–80% of women referred with vulval symptoms have at least one positive
result on patch testing.
36. Ultrapotent steroids are important in the management of women
diagnosed with lichen sclerosus and lichen planus. { C }
Corticosteroids have antiinflammatory and immunosuppressive
properties .
Clobetasol propionate is the most potent topical corticosteroid
available.
Response rates of women diagnosed with lichen sclerosus are
high.
Improvement in vulval skin texture and colour is seen less often.
Women under the age of 50 years had the highest response rates.
Relapse is common: 84% of women experience a relapse within 4
years.
Higher response rates are seen with longer regular use before
returning to ‘as required’ use.
Clobetasol propionate appears to be effective and safe in
premenarchal girls.17
37. Clobestasol cream/ointment should be applied sparingly (this means
half to one finger tip) to the affected area(s) with itch/discomfort or
changes in the skin
The cream to be applied:
once daily for 1 month then
on alternate days for 1 month then
twice a week for 1 month then
once a week for 1 month then
gradually reduced until could be used it occasionally or
not at all.
One 30 g tube of clobetasol cream should last at least 3 months.This
amount should not cause adverse effects on the treated skin or
elsewhere in the body.
If symptoms keep coming back quickly on stoppage using the cream,
the cream is used regularly once or twice a week long term. Longterm
use is safe as long as one 30 g tube lasts at least 3 months. More than
this may cause skin thinning.
stinging for a few minutes after applying the cream is normal.
However, stinging in the area for more than 1–2 hours after applying
the cream, means hypersensitivity, so change the formula.
38. Approximately 4–10% of women with anogenital lichen sclerosus will have symptoms that
do not improve with topical ultrapotent steroids (steroidresistant disease). { D }
The recommended secondline treatment is topical tacrolimus under the supervision of a
specialist clinic. { D }
Tacrolimus and pimicrolimus belong to the class of immunosuppressant drugs known as
calcineurin inhibitors.
Have both been shown to be effective at controlling a number of vulval dermatoses
including lichen sclerosus and lichen planus.
Maximal effects were seen after 10–24 weeks of treatment
Calcineurin inhibitors are well tolerated and their use avoids the adverse effects of
steroids.
However, the longterm safety of topical calcineurin inhibitors is not established.
While awaiting longterm data, use for longer than 2 years is not recommended owing to
concerns about potential malignant transformation.
A number of other oral and topical therapies for secondline treatment have been reported
in small case series, but there is not sufficient evidence to recommend these agents at
present.
Surgery and CO2 laser vaporisation are not recommended for the treatment of symptoms
of lichen sclerosus. However, these treatments have a role in restoring function
impaired by agglutination and adhesions such as urinary retention or narrowing of
the vaginal introitus that affect sexual function or body image.
{ D }
39.
40. Washing with water only causes dry skin and makes itching worse.A soap substitute to be used to
clean the vulval area.( small amount of the cream or ointment with water ).
Shower rather than bath and clean the vulval area only once a day. Overcleaning can aggravate
vulval symptoms.
Avoidance of using sponges or flannels to wash the vulva.The vulval area to be dried with a soft
towel.
Wear loosefitting cotton clothes.
Avoid fabric conditioners and biological washing powders.
Avoid soaps, shower gel, scrubs, bubble baths, deodorants, baby wipes or douches in the vulval
area.
Some overthecounter creams including baby or nappy creams, herbal creams may include possible
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 colours of underwear. Dark textile dyes (black, navy) may cause an allergy
Avoid coloured toilet paper.
Emollients can be used as moisturisers throughout the day.Using one of these moisturisers every
day can help relieve symptoms. Even when you do not have symptoms, using a moisturiser will
protect the skin and can prevent flareups.
If your skin is irritated, aqueous cream can be kept in the fridge and dabbed on to cool and soothe
the skin as often as you like.
41. Pruritis
Vulval pain
Change in colour & texture of the skin
Vulval ulcer
Bullous & blistering disorders
Vulval lumps (cystic, nodule or papule)
43. -Present with irritation and
soreness of the vulva and anus
rather than discharge.
-Diabetes, obesity and
antibiotic use may be
contributory.
-Vulval candidiasis may become
chronic and a leading edge of
inflammation with satellite
lesions extending out from the
labia majora to the inner thighs
or mons pubis.
-Prolonged topical antifungal
therapy may be necessary to
clear a skin infection with oral
or topical preparations.
45. -Occurs in skin where sebaceous
glands are active e.g. face
scalp&genitalia.
-Labia majora & mons pubis are
affected
-The lesions are itchy, scaly, poorly
demarcated, orange pink in colour
-A long history of intermittent
dandruff.
-It is associated with Malassezia
Ovalis infection (a commensal lipo-
philic yeasts).
-Treatment with antifungal(2%
Miconazole or 2%ketoconazole
cream or shampoo)+low dose mild
to mid-potency topical steroids
twice daily for 1-2 weeks then a low
dose topical steroid and imidazole
cream for maintenance.
46. -Is a rare vulval condition
-seen in postmenopausal women.
-The main symptom is pruritus.
-Lesions have a florid eczematous
appearance with lichenification,
erythema and excoriation.
-Can be associated with an
underlying adenocarcinoma.
- The gastrointestinal and urinary
tracts and the breasts should be
checked.
- Surgical excision is recommended
to exclude adenocarcinoma of a skin
appendage.
-Photodynamic therapy and topical
imiquimod have been used with
some success.
- Despite obvious clinical features,
surgical margins are difficult to
achieve owing to subclinical
disease, and recurrence is common.
47. 1. VIN, usual type
VIN, warty type
VIN, basaloid type
VIN, mixed (warty/basaloid) type
2. VIN, differentiated type
48. -Nearly all VIN is of usual type
-Is more common in women aged
35–55
-It is associated with HPV
(especially HPV16) CIN, VaIN, ,
cigarette smoking and chronic
immuno-suppression.
-It may be multifocal and multi
centric.
-The appearance varies widely:
red, white or pigmented; plaques,
papules or patches; erosions,
nodules, warty or hyperkeratosis.
-Usual type VIN is associated with
warty or basaloid squamous cell
carcinoma
49. -Is rarer than usual type
-Is seen in older women aged
55–85.
-Some cases are associated
with lichen sclerosus.
Is not related to HPV and does
not appear to have a long
intraepithelial stage.
- It is linked to keratinising
squamous cell carcinomas of the
vulva.
-Clinically, it tends to be uni-
focal in the form of an ulcer or
plaque.
-The risk of progression appears
to be higher than in usual type
VIN.
-The symptom of pruritus can
be intractable, although the use
of emollients or a mild topical
steroid may help.
50. The gold standard for the treatment of VIN is local surgical excision.{ C }
Women undergo treatment of VIN to :
1. relieve symptoms of severe pruritus,
2. exclude invasive disease and
3. reduce the risk of developing invasive cancer.
Simple and radical vulvectomy : inappropriate owing to their adverse effects on
sexual function and body image.
Local excision: is adequate with the same recurrence rates and provides a
specimen for histological diagnosis. 12 to 17 % of women undergoing excision of VIN
have clinically unrecognised invasion diagnosed on histology.
If surgical treatment is not undertaken, adequate biopsy sampling is required to
reduce the risk of unrecognised invasive disease.
Complete response rates are higher with excision than with ablative or medical ttt
Women undergoing surgical excision of VIN should have access to reconstructive
surgery. { D }
Nonsurgical treatments are accepted as an alternative to surgery, but women
require regular, longterm followup { B }
1. Topical imiquimod cream
2. Cidofovir
3. Laser ablation
4. cavitron ultrasonic surgical aspiration, photodynamic therapy, interferon and
therapeutic human papillomavirus (HPV) vaccine,
51. Vulvar Pain Related to a Specific Disorder
• 1) Infectious (e.g. candidiasis, herpes, etc.)
• 2) Inflammatory (e.g. lichen planus, immunobullous disorders,
etc.)
• 3) Neoplastic (e.g. Paget’s disease, squamous cell carcinoma,
etc.)
• 4)Neurologic (e.g. herpes neuralgia, spinal nerve
compression, etc.)
Vulvodynia
• 1) Generalized
a) Provoked (sexual, nonsexual, or both)
b) Unprovoked
c) Mixed (provoked and unprovoked)
• 2) Localized (vestibulodynia, clitorodynia, hemivulvodynia, etc.)
a) Provoked (sexual, nonsexual, or both)
b) Unprovoked
c) Mixed (provoked and unprovoked)
53. Behcet’s disease (multiple ,painful)
Crohn’s disease (multiple ,painful)
Hydradinitis suppuritiva (multiple , painful, on
top of nodule)
Herpes simplex (multiple ,painful ,arise on top
of vesicles)
Syphilis (solitary ,not tender, arise from papule)
Chancroid (arise from papule)
Granuloma Inguinal
Lymphogranuloma Venerium (solitary ,not
tender)
Vulval cancer (solitary ,not tender)
54. -Chronic
,suppurative,inflammatory
disorder of the apocrine glands.
-Primarily affects the labia
majora & inter-crural folds but
may also involve the mons pubis
,labia minora &clitoris.
-Deep painful subcutaneous
nodules that may ulcerate & drain
leading to sinuses & extensive
scarring.
-Common especially in black
women
-Unknown aetiology.
-Multiple therapies have been
used with limited success (topical
& systemic antibiotic ,oral CC
,steroids and isotretinoin) {C}
-Surgery remains the main-stay in
ttt & wide excision may be
necessary.{C}
58. Pruritus vulvae and vulval pain are very common complaints and most
women initially self medicate. Although it is often selflimiting, chronic
vulval pruritus suggests an underlying vulval dermatosis.
Careful and systemic examination is fundamental to making a diagnosis.
Skin biopsies are not always necessary but are essential if VIN or invasive
disease is suspected or if the condition does not respond to treatment.
General care of vulval skin is a fundamental component of
treatment.Avoidance of potential irritants will benefit most conditions.
The mainstay of the management of lichen sclerosus is topical
ultrapotent steroids. Women require clear advice on the appropriate
treatment regimes.
Women with VIN require a biopsy to confirm disease.Longterm
surveillance is necessary, particularly when a medical or conservative
approach to management is taken.
All gynaecological trainees require experience in the management of
common skin disorders, but a specialist service improves care for women
by improving the accuracy of diagnosis and the implementation of
adequate and appropriate treatment.