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Full text of "DR_H_1aim.net"

Vulval Disorders; 

Update 



Prof. Amr El-Shalakany, MD FRCOG. 

Gynecological Oncology Unit 

Early Cancer Detection Unit 

Ain Shams University 

Cairo, Egypt _. 




Vulval Disorders 



Vulval complaints form 5-10% of all 
gynaecological complaints 



Tendency for under reporting and less 
attention from physicians _ 



Can cause extreme distress and may lead to 
marital problems 



Vulval Disorders 



The vulva is a piece of skin, 



Therefore, general dermatologic conditions 
(e.g. eczema, psoriasis, pemphigus, lichei^ 
simplex, lichen planus) must be considered 
when faced with a vulval lesion. 



The vulva 



***It is of paramount importance to involve 
dermatologists and genito-urinary 
physicians where the diagnosis is in doubt. 



*** Always remember that a vulval complaint 
can be a manifestation of a systemic disease 
e.g. diabetes, psoriasis, SLE. 



Systemic diseases 



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Vulval diseases 



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Lichen planus 




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Vulval candidiasis 






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Childhood vulvovaginitis 



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Vulval H. simplex & Zoster 



Vulval MoUscum contagiosum 



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Behcet's syndrome 



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Vulval haemangioma 






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Vulval lymphoma 



Vulval Choriocarcinoma 




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Vulvar lesions 






Fibroepithelial polyp 



Granulomatous Cheilitis 

Melkersson-Rosenthal disease 



Confusing findings 



Indications for coIdoscodv of the vulva: 



1. Changes in skin pigmentation. 

2. Ulcers, warts, nodules,thickening, Hssuring. 

3. Pruritus vulvae, vulval burning, vulvodynia. ^ 

4. Dysuria (in the absence of UTI or calculi). 

5. Follow up of treated VIN, non neoplastic 



disorders or vulval carcinoma. 



Colposcopy of the vulva is more difficult 

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to interpret than that of the cervix or vagina. 



***Keratinization of vulval skin can mask or 
alter the appearance of underlying epithelium 
and vasculature. 



***Therefore, identifying lesions and grading 
their severity is more complex. 



** 



The junction between the glycogen bearing 



vaginal epithelium and the keratinized vulval 
skin is at high risk for developing VIN. 



**If VIN or HPV is suspected, it is obligatory ^ 



to proceed to colposcopic examination of the 



cervix and vagina, including collection of a 
cervical smear for cytology. 



Technique of vulval colposcopv: 



1. Explain the technique to the patient. 

2. Modified Uthotomy position. 

3. Inspection with the naked eye. 

4. Paint the vulva with hydrogel. ^ 

5. Inspection with low power magniHcation 

(6-fold) in a systematic order, ^m 

6. Application of 5 % acetic acid for 3 min. 

then repeat inspection. ^ 



Toluidine blue application is unreliable for 
the detection of lesions.^^ 



Vulval biopsy: 



It is mandatory to confirm the histopatologic 
nature and the severity of the lesion. 



Biopsy techniques are: 

• Excision biopsy (whole lesion is removed) 

• Keye's punch biopsy. 

• Large Knife incision biopsy. 

• Diathermy loop biopsy. 



Vulval biopsy (Keye's punch biopsy) 





Classification of vulvae disease (1987): 



(1) Non neoplastic vulvae epithelial disorders: 
I. lichen sclerosus . 
II. Squamous cell hyperplasia. 
III. Other dermatosis. 



(2) Vulval intraepithelial neoplasia (VIN). 



(3) Human papilloma (HPV) virus infection: 
I. Condyloma accuminata. 
II. Subclinical HPV infection. 



ISSVD Classification of Vulvar 
Dermatoses (2006) : 

• Spongiotic pattern 

- Atopic dermatitis 

- Contact allergic dermatitis 

- Irritant contact dermatitis 

• Acanthotic pattern (formerly 
squamous cell hyperplasia). 

- Psaoriasis 

- Lichen simplex chronicus (primary 
and secondary) 

• Lichenoid 

- Lichen sclerosus 

- Lichen planus 



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ISS VD Classification of Vulvar 
Dermatoses (2006) : 

• Dermal homogenisation / sclerosis 

- Lichen sclerosis 

• Vesiculobolbous pattern 

- Pemphigoid 

- Linear Ig A disease 

• Acantholytic pattern -* 

- Hailey-Hailey disease 

- Darier disease 

- Papular genitocmral acantholysis 



ISS VD Classification of Vulvar 
Dermatoses (2006) : 



Granulomatous 

- Crhon's disease 

- Melkersson-Rosenthal disease 

Vasculopathic pattern 

- Behcet's disease 

- Aphthous ulcer 

- Plasma cell vulvitis 



ISSVD terminology and classification of 
vulvar pain 2003 

• Vulvar pain related to a specific disorder 

- Infectious (e.g. candidiasis..) 

- Inflammatory (e.g. lichen planus. . .) 

- Neoplastic (e.g. carcinoma. . .) 

- Neurologic (e.g. Herpes, nerve compression ^J 

• Vulvodynia (pain not related to a specific disorder) 

- Generalised I 

• Provoked (sexual or non sexual) 

• Unprovoked 

• Mixed i 

- Localised (vestibulodynia, clitorodynia, hemivulvodynia) 

• Provoked 

1 

• Unprovoked 

• Mixed 



Vulvodynia 



Most likely, there is not 
a single cause. 

Embryologic 
abnormalities, 

Increased urinary 
oxalates, 

Genetic or immune 
factors. 

Hormonal factors. 

Inflammation, infection. 

Neuropathic 




Vulvar Care Measures 

Wearing cotton underwear in the daytime and none 
at night. 

Avoiding vulvar irritants (perfumes, dyed toilet 
articles, shampoos, detergents, and douches). J 

Use of mild soaps, with none applied to the vulva. 

The vulva can be cleaned gently with water and r 
patted dry. ^ 

Emollient without preservatives (vegetable oil or 



plain petrolatum) helps to hold moisture in the skin 
and to improve the barrier function, r 



Vulvar Care Measures 



If menstrual pads are irritating, cotton pads may be 
helpful. 

Adequate lubrication for intercourse is 
recommended. m 

Ice packs are helpful in some, but produce irritation 
when overused. 

Cool gel packs may be used. J| 

Rinsing and patting dry the vulva after urination 
may be helpful. _ 

Use of hair dryers should be avoided. 



Vulvodynia 



Vulval care measures 

Topical emolients, steroids, local anaesthetics, 
estrogens 

Oral antidepressants, anticonvulsants, SSRIs 

Low-Oxalate Diet with Calcium Citrate 
Supplementation 

Botulinum toxin 

Nitroglycerine , 

Biofeedback, hypnotherapy, acupuncture 

Vestibulectomy 



Lichen sclerosus (LS) 



One fourth of women seen in vulva clinics 
1/300-1/1000 women affected 
Most cases occur postmenopausally 
Can occur in young women 

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Equivalent to balanitis xerotica obliterans 
Remittent course 

Can affect non genital skin in 20% 
Autoimmune ? Hormonal ? , 



Lichen sclerosus 



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Lichen sclerosus 



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Lichen sclerosus 



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Lichen sclerosus (LS) 

• Pruritus is the main symptom 

• (3 % ) associated with vulval carcinoma 

• Management is essentially with clobetasol 

• Macrolide immunosuppressants (e.g 
Tacrolimus) 

• Surgery is rarely required J 

• Long term follow up with specialist "referral 



quamous cell hyperplasia 



(Lichen simplex planus) 
(Hyperplastic dystrophy) 

Pruritus plus wide range of 
skin change. 

Biopsy is essential esp. 
hyperkeratotic areas 

Long term follow up is needed 

Treatment with steroids 





quamous cell hyperplasia 






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Squamous cell hyperplasia 




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Breaking the itch-scratch cycle is fundamental to the 
treatment of lichen simplex chronicus (Antihistaminics, 
Tricyclic antidepressants, SSRIs). 

Steroids ^ 

Treatment of chronic irritative infections 



VIN ISSVD classification 2004 



VIN 1 is no longer included 

VIN2 & VIN3 amalgamated in one category 

1. VIN, usual type (HPV related) 

• VIN, warty 

• VIN, basaloid J 

• VIN, mixed I 

2. VIN, differentiated type (Non- HPV 
related) 



VIN 1 now = subclinical HPV 





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Vulval intraepithelial neoplasia (VIN) 



Atypia involving the sq. epith. 
Grades according to thickness involved 
Presence of koilocytosis is not an element 
60% involve non hairy skin j 

66% multifocal and 33% unifocal ^ 
Pruritus, soreness, lump or asymptomatic - 
Biopsy & histology are essential ■ 



VIN & vulval Paget' s 





Vulval intraepithelial neoplasia (VIN) 



Younger women usually have multifocal 
disease 

43-79% of VIN lesions show HPV 

Risk of invasive disease is obscure (5%)? J 

25-33% of invasive disease show VIN3 



Risk of invasion is usually postmenopausal 



Vulval intraepithelial neoplasia (VIN) 





^■'- 





Vulval intraepithelial neoplasia (VIN) 

• Management involves long term follow up 

• Up to 84% recurrence after surgery even in 
grafted skin 

• Biopsy , exclude invasion, expectancy and 
avoid mutilating surgery 

• Treat postmenopausal & immunosuppressed 

• Vulvectomy, skinning vulvectomy, WLE, ^ 
Laser, DNCB, 5-FU, INF, photodynamic ^ 
therapy , 

• Immiquimod van set ersetalNEngU Med 2008;358:1465-73. 



Vulval Paget ^s 

• Sharply demarcated brick 
red, scaly, eczematoid 
plaque 

• 26 % non vulval 
adenocarcinoma and 4 % 
vulval adenocarcinoma. 



Workup should include 
colonoscopy, cystoscopy, 
mammogram, and 
colposcopy. 



Vulval Paget ^s 



Therapy involves excision 
with a 2-3 cm safety 
margin. 

31% to 43% local 
recurrence rate 

Laser ablation is not 
appropriate (need to 
achieve deep tissue 
destruction). 

Prognosis is determined by 
the nature of the coexisting 
adenocarcinoma, if 
present. 



HPV Condylomata Acuminata 




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HPV Condylomata Acuminata 



External genital warts caused by 
HPV 6, 11, 42, 43, 44 

Exophytic benign lesions 

Can cause hyperkeratotic diffuse 
skin thickening 

Treatment is only for cosmetic 



purpose 



HPV and condyloma accuminata 
^ [Genital Warts (GW)] 

The UK National Survey of Sexual Attitudes and 
Lifestyles conducted in the year 2000 



• 3.6% of men had Genital warts 

• 4. 1 % of women 

• The most common reported sexually 
transmitted infection. 

• Increase over years. ^ 

Fenton et al. Lancet 2001;358(9296):1851-4. 



Number of diagnoses of genital warts (first, recurrent and 
reregistered episodes) by sex, STD clinics: England and 
Wales 1971-2004. 



70,000 



CO 60,000 

LU 

CO 

Q 50.000- 



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^ 40,000 H 



p 30.000 - 

LU 

CQ 20.000 H 



10.000 



— Male 

— Female 




-i — r — I — 1 — r 



■7 — f — I — I — i — I — i — f — f- 



-r — i — r 





1971 1974 1977 1980 1983 1986 1989 1992 1995 1998 2001 2004 

YEARS 



HPV genital warts burden 

The cost of a single successful episode of 
treatment of a case of GWs to be 



- $ 377 in the UK ($ 54 million per annum) 

- $ 436 in the USA. ($ 200 million per annum) 



Langely et al, IntJSTD AIDS 2004; 15(8):50 1-8. 
Insinga etal., Phannacoeconomics 2005;23(1 1):1 107-22. 



Beware of vestibular papillomatosis 






HPV Condylomata Acuminata 



Cervical Cytology and 
colposcopy are 
required 



Screen for other STD 



Husband or partner 



screening 




■-<^~^-^^ 




HPV Condylomata Acuminata 

Treatment 



Podophyllin 


22-80% 


BCA and TCA 


64-80% 


5FU 


50-90% 


Cryocautery 


70-96% 


Diathermy 


72-94%) 


C02 Laser 


72-97 % 


Surgical excision 


89-93% 


Systemic interferon 


25-35% 


Intralesional interferon 


^ 36-52% 


Imiquimod J 


H 50% 



HPV Condylomata Acuminata 
Treatment 

Imiquimod5% 



r.v 



Aldara cream 



V:X ^ 



Immune response modifier 

3 applications weekly for 
12-16 week 



50% success rate 



HPV Condyloma Acuminata 



Can be treated between 14-32 wks 

Cytotoxic medications can not be used ^ 

Caesarean section doses not alter the risk of 
vertical transmission 

Caesarean section only when the vagina is 
involved with multiple vascular growths 



Vulval Cancer 

4-5% of genital malignancy 

• 90 % squamous cell 
carcinoma 

- HPV or VIN associated 

(25-33%) 

-Chronic inflammatory 
process related 

• 2 % basal cell carcinoma 

• 2-8% melanomas 



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Vulval cancer 





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Squamous cell carcinoma of the vulva 





FIGO Staging 



Stage I: Tumor confined to the vulva 

- lA Lesions <2 cm in size, confined to the vulva or 
perineum and with stromal invasion <1 .0 mm, no 
nodal metastasis i 

- IB Lesions >2 cm in size or with stromal invasion >1 .( 
mm, confined to the vulva or perineum, with negative 
nodes ^^ 

Stage II: Tumor of any size with extension to adjacent 
perineal structures (1/3 lower urethra, 1/3 lower vagina, 
anus) with negative nodes ■ 



FIGO Staging 



Stage III: Tumor of any size with or without extension to 
adjacent perineal structures (1/3 lower urethra, 1/3 lower 
vagina, anus) with positive inguino-femoral lymph nodes 



(ii) 1-2 lymph node metastasis(es) (5 mm) 

- NIB (i) With 2 or more lymph node metastases (>5 mm), or 

(ii) 3 or more lymph node metastases (<5 mm) 

- NIC With positive nodes with extracapsular spread 



FIGO Staging 



Stage IV: Tumor invades other regional (2/3 upper urethra, 
2/3 upper vagina), or distant structures 



- IVA: Tumor invades any of the following: . 

• (i) upper urethral and/or vaginal mucosa, bladder 
mucosa, rectal mucosa, or fixed to pelvic bone, or 

• (ii) fixed or ulcerated inguino-femoral lymph nodes 

- IVB: Any distant metastasis including pelvic lymph 
nodes 



Squamous cell carcinoma of the vulva 



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Vulval cancer 



Basset's Radical vulvectomy (wound 
breakdown, TE, lymphoedema) 

Wide local excision and hemivulvectomy 

Split incisions for inguinal node dissection 

Sentinel lymph node technique -^ 

Multimodal therapy and reconstruction for 
recurrent or advanced disease 



Vulval cancer 








Conservatism in Vulvar Cancer 




This is wiiat we do now 



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Conservatism in Vulvar Cancer 



{Radical vulvectomy is associated with wound break down, 



lymphoedema, DVT & TE, mutilation ) 

• Hemivulvectomy + unilateral LND 

• Split incisions (Vs Basset's Butterfly incision) 

• Sentinel lymph node 
Endoscopic inguinal LND 
Reconstructive surgery 



Conclusion 



Vulval complaints are not uncommon. 



Vulval complaints can markedly affect 
patient's quality of life j 



Vulval disorders deserve better attention 
from gynecologists 



Conclusion 



Involvement of dermatologists and 
genito urinary physicians are 
important. 

Sub specialist opinion (gynecological 
oncologist) is essential in the 
management of premalignant and 
malignant vulval lesions 



Acknowledgement 

Faculty members, doctors, nursing 
staff, technicians and workers of 



Early Cancer Detection Unit^ 



Gynaecological Oncology Unit 



Ain Shams University 



I