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Diseases of vulva
Associate Clinical. Prof. Dr Aisha M EL- Bareg, MD, PhD
Senior Consultant Obs & Gyn / Reproductive. Med
Faculty of Medicine, Misurata University, Libya
External genitalia
Anatomical consideration
• Vulvar skin comprises stratified squamous
epithelium as in other parts of body.
• The mons pubis and labia majora contain fat,
sebaceous, apocrine and eccrine sweat glands and
blood vessels , which can develop varicosities.
• Labia minora are rich in sebaceous glands, contain
few sweat glands but no hair follicles.
• The epithelium of the vestibule is neither
pigmented nor keratinized, but contain eccrine
glands. These glands and epithelial appendages are
source of lumps
Benign Vulval lumps
• Bartholin’s cyst.
• Epidermal inclusion cyst.
• Skene’s duct cyst.
• Congenital mucous cysts: arise from
mesonephric ducts remnants.
• Cyst of the canal of Nuck: can give rise to
hydrocele in labia majora.
• Sebaceous cyst.
• Papillomatosis (solid).
• Fibroma (solid).
• Lipoma (solid).
• Condylomata (solid).
Cysts are either congenital or arise from
obstructed glands.
Manifestations arise from the cysts (cosmetic)
or from infection.
Benign Vulval lumps
Bartholin’s Cyst
• Bartholin’s glands situated in posterior part of the
labia. Normally not seen nor felt
• If enlarged, can be a painless cyst or painful
abscess
• Lymphatics drain to inguinal nodes
• Secrete mucus, particularly
during intercourse
• Can block causing retention cyst and if
superimposed with infection-an abscess.
• Treated surgically by marsupialization
Bartholin’s Cyst
Don’t Confuse it with:
Inclusion Cyst of the Vulva Right Vaginal Wall Cyst
Skene's Gland
• Each side of urethra
• Normally neither seen
nor felt
• May become swollen
and tender, particularly
with GC or chlamydia
• Culture, I&D if pointing
Non-neoplastic epithelial disorders
Classification:
1. Lichen sclerosis.
2. Squamous cell hyperplasia (formerly:
hyperplastic dystrophy).
3. Other dermatoses.
- lichen planus.
- psoriasis.
- seborrhoeic dermatitis
- inflammatory dermatoses.
- ulcerative dermatoses.
Lichen sclerosus
• Etiology: unknown
• Can affect both sexes and at any
age
• Typically found in anogenital
region in postmenopausal women
• It can be a symptomatic
Comprises 70% of benign epithelial disorders →
epithelial thinning, inflammation & distinctive
histological changes in the dermis.
• Sx: intractable itching (commonest), vaginal soreness +
Dyspareunia.
• Signs: crinkled skin, L. minora atrophy, constriction of
V. orifice, adhesions, ecchymosis & fissures.
• Dx: Biopsy is mandatory
• Rx: emollients, topical steroids.
- Testosterone: not effective than petroleum jelly & →
pruritus, pain & virilization.
- Surgery: avoided unless malignant changes
Lichen sclerosus
Lichen Planus
• General Appearance
–Erosive lesions at vestibule w/without adhesions
resulting in stenosis
–May have associated oral mucotaneous lesions
and desquamated vaginitis
–Patient c/o irritating vaginal , vulvar soreness,
intense burning, pruritus, and dyspareunia
w/post-coital bleeding
–Types: Papulosquamous LP/ Hypertrophic LP and
Erosive LP
Lichen Planus
• The lesions tend to disappear after weeks or months
• Erosive lesions heal poorly and may be
pre-malignant .
• Diagnosis: is confirmed by biopsy
• Treatment
• Topical steroid, Vaginal estrogen cream if atrophic
epithelium, Vaginal dilators for stenosis
–Surgery for severe vaginal synechiae
–Vulvar hygiene
–Emotional support
Vulval Psoriasis
• Physical Appearance
–Red moist lesions
w/without scales
• Treatment: Topical
corticosteroids
Benign epithelial thickening and hyperkeratosis
◦Acute phase with red/moist lesions
◦Causing pruritus leading to rubbing & scratching
◦Circumscribed, single or unifocal
◦Raised white lesions on vulva or labia majora and
clitoris
Treatment: Sitz baths, lubricants, oral
antihistamines, Medium potency topical steroid
twice daily
Squamous Cell Hyperplasia
(Atopic Eczema/ Neurodermatitis)
Inflammatory dermatomes
• Can be classified as either:
- contact dermatitis - primary irritant dermatitis
• It is difficult to differentiate between the two
• Typical findings are:
diffuse reddening of the involved skin with
excoriation and ulceration. Secondary infection
may occur.
• D.D: vulvar candidiasis
Inflammatory dermatomes
• Etiology: Local irritants as perfumed soap,
deodorant, bubble baths, tight clothing, and urine
• The incidence is unknown
Treatment
avoid local cause
oral antihistamine
topical corticosteroid
Seborrhoeic dermatitis
• Occurs in areas of the skin where sebaceous glands
are active, such as face, body folds, and less
common genitalia
• The common sites of the vulva are labia majora and
mons pubis
• The lesions are scaly, orange pink in color and can
be secondarily infected
• It is uncommon vulvar problem
• Treatment: Antifungal as miconazole or
ketoconazole cream
Ulcerative dermatomes
• The ulcerating lesions may be solitary or multiple,
painful or non-tender
• The lesions are uncommon
• Etiology
• Herpes simplex virus which are vesicle then ulcer
• Syphilis are papule and then ulcerate
• Cancroids, granuloma inguinale, and
lymphgranulma venerum
• Diagnosis: Serology, Culture
• Treatment
• According to the cause
Genital warts
Condylomata accuminata
• Are caused by HPV. May involve not only the vulval
skin but also the vagina and cervix
• There are more than 50 types of HPV, most
important type 6,11,18
• Typically lesions are elevated, discrete but
sometimes confluent and covering large area
• Tends to increase in size in patients using COP and
during pregnancy
• The disease transmitted sexually
• Diagnosis confirmed histopathologically
• Treatment
–25% trichloroacetic acid (TCA), Podophyllin
–Podophyllin should not be used during pregnancy
–If resistant to podophyllin
• liquid nitrogen application, cryosurgery,
electro diathermy, Carbon dioxide laser
• Interferon
Genital warts
Condylomata accuminata
(Vulvar Intra-epithelial Neoplasia (VIN)
 Neoplastic cells are confined to the surface
epithelium of the vulva
 It is a premalignant condition < CIN
 The major factor is HPV (type 16, and 33)
 Incidence of invasive carcinoma 4%.
 VIN affects mainly labia minora and perineum
but may extend to peri anal area
 May persist for longer periods of LP > 10 years
• May be asymptomatic but can cause:
• Pruritus-soreness, burning (white lesions}
Vulvar Intra-epithelial Neoplasia (VIN)
Histological Classification of VIN
1. Squamous Intra-epithelial Neoplasia (SIN)
• VIN 1 (lower 1/3 of vulvar epithelium)
• VIN 2 (lower 2/3 of vulvar epithelium)
• VIN 3 (full thickness of the vulvar epithelium)
2. Non-squamous Intra-epithelial Neoplasia
• Paget’s disease
• Tumors of the melanocyte
• Diagnosis: Colposcopy and biopsy
• Treatment: depends on age and extent
wide local excision
skinning vulvectomy
simple vulvectomy
Carbon dioxide laser Rx
5% 5-fluoro-uracil ointment
Carcinoma of the Vulva
• Uncommon, accounts for app. 1-4% all female
genital malignancy.
• Seen in elderly women, Average age of 60 years
• Etiology: UNKNOWN
–Herpes simplex virus type 2
–HPV types 16, 18, 31
–Genetic factors
–Coal tar containing chemicals
–Impairment of the immune system
• Primary tumor
–90% squamous cell carcinoma
–Melanoma
–Sarcoma
–Basal cell carcinoma
–Bartholin’s gland carcinoma
–Verrucous carcinoma
Secondary tumor
• It is occasionally found in vulva
• The primary is commonly from the Cx and
endometrium.
Carcinoma of the Vulva- types
• Free of symptoms
• Pruritus vulvae or irritation of the vulva
• Hyperpigmented, a white or red lesion
• Ulcerative or tumor as late lesions
• Spread is mainly lymphatic and also direct
Carcinoma of the Vulva- clinical pictures
• The labia majora is the most common site of
involvement and accounts for about 50% of cases.
• The labia minora accounts for 15% to 20% of cases.
• The clitoris and Bartholin’s glands are less
frequently involved.
Carcinoma of the Vulva- Site
• Stage 0 VIN or CIS of the vulva
• Stage I Tumor confined to the vulva and/or perineum < 2 cm
in greatest dimension, No lymphnode
• Stage II Tumor confined to the vulva and/or perineum > 2 cm
in greatest dimension, No lymphnode
• Stage III Tumor of any size with:
i. Spread to the lower urethra and/or the vagina, anus and/or
ii. Unilateral regional lymphnode metastasis
• Stage IV A Tumor invasion: upper urethra, bladder mucosa, rectal
mucosa, pelvic bones and/or regional lymphnode
• Stage IVB Any distant metastasis and /or the pelvic nodes
Carcinoma of the Vulva- FIGO staging
Risk factors for node metastasis
• clinical node status,
• age,
• degree of differentiation,
• tumor stage,
• tumor thickness,
• depth of stromal invasion, and
• presence of capillary-lymphatic space invasion
Special Investigations
• A biopsy with histological confirmation
• A full blood count, Urea and electrolytes, Liver
function tests
• Colposcopy in early lesions, Chest X-ray
• FNA of the lymph nodes
• Urethrocystoscopy, Proctoscopy
• IVP, MRI, CTS
Treatment of Vulval Carcinoma
Stage I & II :
Radical local excision with 1cm disease–free margin.
Stage III & IV :
- According to the general health.
- Chemotherapy & radiotherapy to shrink the tumor
to permit surgery which may preserve the urethral &
anal sphincter function.
- Radical vulvectomy + inguinal L. nodes dissection.
- Reconstructive surgery with skin grafts or
myocutaneous flaps for healing.
Complications of Radical Vulvectomy
• Femoro-inguinal lymphocysts
• Leg oedema
• Wound breakdown
• Thromboembolism
• Wound infection
• Secondary hemorrhage
Diseases of the Vulva: A Guide for Clinicians

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Diseases of the Vulva: A Guide for Clinicians

  • 1. Diseases of vulva Associate Clinical. Prof. Dr Aisha M EL- Bareg, MD, PhD Senior Consultant Obs & Gyn / Reproductive. Med Faculty of Medicine, Misurata University, Libya
  • 3. Anatomical consideration • Vulvar skin comprises stratified squamous epithelium as in other parts of body. • The mons pubis and labia majora contain fat, sebaceous, apocrine and eccrine sweat glands and blood vessels , which can develop varicosities. • Labia minora are rich in sebaceous glands, contain few sweat glands but no hair follicles. • The epithelium of the vestibule is neither pigmented nor keratinized, but contain eccrine glands. These glands and epithelial appendages are source of lumps
  • 4. Benign Vulval lumps • Bartholin’s cyst. • Epidermal inclusion cyst. • Skene’s duct cyst. • Congenital mucous cysts: arise from mesonephric ducts remnants. • Cyst of the canal of Nuck: can give rise to hydrocele in labia majora. • Sebaceous cyst. • Papillomatosis (solid). • Fibroma (solid).
  • 5. • Lipoma (solid). • Condylomata (solid). Cysts are either congenital or arise from obstructed glands. Manifestations arise from the cysts (cosmetic) or from infection. Benign Vulval lumps
  • 6. Bartholin’s Cyst • Bartholin’s glands situated in posterior part of the labia. Normally not seen nor felt • If enlarged, can be a painless cyst or painful abscess • Lymphatics drain to inguinal nodes • Secrete mucus, particularly during intercourse
  • 7. • Can block causing retention cyst and if superimposed with infection-an abscess. • Treated surgically by marsupialization Bartholin’s Cyst
  • 8. Don’t Confuse it with: Inclusion Cyst of the Vulva Right Vaginal Wall Cyst
  • 9. Skene's Gland • Each side of urethra • Normally neither seen nor felt • May become swollen and tender, particularly with GC or chlamydia • Culture, I&D if pointing
  • 10. Non-neoplastic epithelial disorders Classification: 1. Lichen sclerosis. 2. Squamous cell hyperplasia (formerly: hyperplastic dystrophy). 3. Other dermatoses. - lichen planus. - psoriasis. - seborrhoeic dermatitis - inflammatory dermatoses. - ulcerative dermatoses.
  • 11. Lichen sclerosus • Etiology: unknown • Can affect both sexes and at any age • Typically found in anogenital region in postmenopausal women • It can be a symptomatic Comprises 70% of benign epithelial disorders → epithelial thinning, inflammation & distinctive histological changes in the dermis.
  • 12. • Sx: intractable itching (commonest), vaginal soreness + Dyspareunia. • Signs: crinkled skin, L. minora atrophy, constriction of V. orifice, adhesions, ecchymosis & fissures. • Dx: Biopsy is mandatory • Rx: emollients, topical steroids. - Testosterone: not effective than petroleum jelly & → pruritus, pain & virilization. - Surgery: avoided unless malignant changes Lichen sclerosus
  • 13. Lichen Planus • General Appearance –Erosive lesions at vestibule w/without adhesions resulting in stenosis –May have associated oral mucotaneous lesions and desquamated vaginitis –Patient c/o irritating vaginal , vulvar soreness, intense burning, pruritus, and dyspareunia w/post-coital bleeding –Types: Papulosquamous LP/ Hypertrophic LP and Erosive LP
  • 14. Lichen Planus • The lesions tend to disappear after weeks or months • Erosive lesions heal poorly and may be pre-malignant . • Diagnosis: is confirmed by biopsy • Treatment • Topical steroid, Vaginal estrogen cream if atrophic epithelium, Vaginal dilators for stenosis –Surgery for severe vaginal synechiae –Vulvar hygiene –Emotional support
  • 15.
  • 16. Vulval Psoriasis • Physical Appearance –Red moist lesions w/without scales • Treatment: Topical corticosteroids
  • 17. Benign epithelial thickening and hyperkeratosis ◦Acute phase with red/moist lesions ◦Causing pruritus leading to rubbing & scratching ◦Circumscribed, single or unifocal ◦Raised white lesions on vulva or labia majora and clitoris Treatment: Sitz baths, lubricants, oral antihistamines, Medium potency topical steroid twice daily Squamous Cell Hyperplasia (Atopic Eczema/ Neurodermatitis)
  • 18. Inflammatory dermatomes • Can be classified as either: - contact dermatitis - primary irritant dermatitis • It is difficult to differentiate between the two • Typical findings are: diffuse reddening of the involved skin with excoriation and ulceration. Secondary infection may occur. • D.D: vulvar candidiasis
  • 19. Inflammatory dermatomes • Etiology: Local irritants as perfumed soap, deodorant, bubble baths, tight clothing, and urine • The incidence is unknown Treatment avoid local cause oral antihistamine topical corticosteroid
  • 20. Seborrhoeic dermatitis • Occurs in areas of the skin where sebaceous glands are active, such as face, body folds, and less common genitalia • The common sites of the vulva are labia majora and mons pubis • The lesions are scaly, orange pink in color and can be secondarily infected • It is uncommon vulvar problem • Treatment: Antifungal as miconazole or ketoconazole cream
  • 21. Ulcerative dermatomes • The ulcerating lesions may be solitary or multiple, painful or non-tender • The lesions are uncommon • Etiology • Herpes simplex virus which are vesicle then ulcer • Syphilis are papule and then ulcerate • Cancroids, granuloma inguinale, and lymphgranulma venerum • Diagnosis: Serology, Culture • Treatment • According to the cause
  • 22. Genital warts Condylomata accuminata • Are caused by HPV. May involve not only the vulval skin but also the vagina and cervix • There are more than 50 types of HPV, most important type 6,11,18 • Typically lesions are elevated, discrete but sometimes confluent and covering large area • Tends to increase in size in patients using COP and during pregnancy • The disease transmitted sexually
  • 23. • Diagnosis confirmed histopathologically • Treatment –25% trichloroacetic acid (TCA), Podophyllin –Podophyllin should not be used during pregnancy –If resistant to podophyllin • liquid nitrogen application, cryosurgery, electro diathermy, Carbon dioxide laser • Interferon Genital warts Condylomata accuminata
  • 24. (Vulvar Intra-epithelial Neoplasia (VIN)  Neoplastic cells are confined to the surface epithelium of the vulva  It is a premalignant condition < CIN  The major factor is HPV (type 16, and 33)  Incidence of invasive carcinoma 4%.  VIN affects mainly labia minora and perineum but may extend to peri anal area  May persist for longer periods of LP > 10 years
  • 25. • May be asymptomatic but can cause: • Pruritus-soreness, burning (white lesions} Vulvar Intra-epithelial Neoplasia (VIN)
  • 26. Histological Classification of VIN 1. Squamous Intra-epithelial Neoplasia (SIN) • VIN 1 (lower 1/3 of vulvar epithelium) • VIN 2 (lower 2/3 of vulvar epithelium) • VIN 3 (full thickness of the vulvar epithelium) 2. Non-squamous Intra-epithelial Neoplasia • Paget’s disease • Tumors of the melanocyte
  • 27. • Diagnosis: Colposcopy and biopsy • Treatment: depends on age and extent wide local excision skinning vulvectomy simple vulvectomy Carbon dioxide laser Rx 5% 5-fluoro-uracil ointment
  • 28. Carcinoma of the Vulva • Uncommon, accounts for app. 1-4% all female genital malignancy. • Seen in elderly women, Average age of 60 years • Etiology: UNKNOWN –Herpes simplex virus type 2 –HPV types 16, 18, 31 –Genetic factors –Coal tar containing chemicals –Impairment of the immune system
  • 29. • Primary tumor –90% squamous cell carcinoma –Melanoma –Sarcoma –Basal cell carcinoma –Bartholin’s gland carcinoma –Verrucous carcinoma Secondary tumor • It is occasionally found in vulva • The primary is commonly from the Cx and endometrium. Carcinoma of the Vulva- types
  • 30. • Free of symptoms • Pruritus vulvae or irritation of the vulva • Hyperpigmented, a white or red lesion • Ulcerative or tumor as late lesions • Spread is mainly lymphatic and also direct Carcinoma of the Vulva- clinical pictures
  • 31. • The labia majora is the most common site of involvement and accounts for about 50% of cases. • The labia minora accounts for 15% to 20% of cases. • The clitoris and Bartholin’s glands are less frequently involved. Carcinoma of the Vulva- Site
  • 32.
  • 33.
  • 34.
  • 35.
  • 36. • Stage 0 VIN or CIS of the vulva • Stage I Tumor confined to the vulva and/or perineum < 2 cm in greatest dimension, No lymphnode • Stage II Tumor confined to the vulva and/or perineum > 2 cm in greatest dimension, No lymphnode • Stage III Tumor of any size with: i. Spread to the lower urethra and/or the vagina, anus and/or ii. Unilateral regional lymphnode metastasis • Stage IV A Tumor invasion: upper urethra, bladder mucosa, rectal mucosa, pelvic bones and/or regional lymphnode • Stage IVB Any distant metastasis and /or the pelvic nodes Carcinoma of the Vulva- FIGO staging
  • 37. Risk factors for node metastasis • clinical node status, • age, • degree of differentiation, • tumor stage, • tumor thickness, • depth of stromal invasion, and • presence of capillary-lymphatic space invasion
  • 38. Special Investigations • A biopsy with histological confirmation • A full blood count, Urea and electrolytes, Liver function tests • Colposcopy in early lesions, Chest X-ray • FNA of the lymph nodes • Urethrocystoscopy, Proctoscopy • IVP, MRI, CTS
  • 39. Treatment of Vulval Carcinoma Stage I & II : Radical local excision with 1cm disease–free margin. Stage III & IV : - According to the general health. - Chemotherapy & radiotherapy to shrink the tumor to permit surgery which may preserve the urethral & anal sphincter function. - Radical vulvectomy + inguinal L. nodes dissection. - Reconstructive surgery with skin grafts or myocutaneous flaps for healing.
  • 40. Complications of Radical Vulvectomy • Femoro-inguinal lymphocysts • Leg oedema • Wound breakdown • Thromboembolism • Wound infection • Secondary hemorrhage