ALL

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“ Vulavr and vaginal diseaes”
Preti E*, Sideri M*
*Preventive Gynecology Unit

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Anatomical consideration
Vulval 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 gland, contain
few sweat gland but no hair follicles
The epithelium of the vestibule is neither pigmented
nor keratinized, but contain eccrine glands.
These glands and epithelial appendages are a
source of lump

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Infection vulvar lesions
• Herpes simplex/zoster
• HPV
• Chancroid, Lymphogranuloma venereum, syphylis
• Bacterial infection
• Amoebiasis
• Epstein Barr virus
• Histoplasmosis, tubercolosis actinomicesis

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Benign Vulval lumps
• Bartholin’s cyst.
• Epidermal inclusion cyst.
• Skene’s duct cyst.
• Congenital mucous cysts: arise from mesonephric ducts remnante.
• Cyst of the canal of Nuck: can give rise to hydrocele in labia maqjora.
• Sebaceous cyst.
• Papillomatosis (solid).
• Fibroma (solid).
• Lipoma (solid).
 Cysts are either congenital or arise from obstructed glands.
 Manifestations arise from the cysts (cosmetic) or from infection.

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Case Report
2007 Lipoma (solid)
S. M 30 years old
2006 Pain and edema of
left labia majora
2006 Incision of the Bartholin’s cyst
- - biopsy - - > adipose tissue

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Non-neoplastic epithelial disorders
• Lichen sclerosis.
• Lichen planus.
• Other common inflammatory dermatoses (lichen simplex,
vulvar eczema, vulvar psoriasis)
• Squamous cell hyperplasia (formerly: hyperplastic
dystrophy).
• Ulcerative dermatoses ( pemphigus , bullous pemphigoid,
Hailey- Hailey disease, apthous ulceration, chron’s
disease, Behcets disease)
• Seborrhoeic dermatitis

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The diagnosis of vulvar diseases presents an enigma.
• For many years there was no classification of these
diseases
• Various clinical specialists (gynecologists,
dermatologists, proctologists,and gastroenterologists)
dealing with vulvar lesions
• Benign vulvar lesions are various and correlate with
dermatological – inflammatory – infection diseases

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WHITE LESIONS
leukoplakia
kraurosis vulvar
dystrophies chronic
atrophic vulvitis
hypertrophic vulvitis
vulvar itthyosis
lichen sclerosus
Squamous intraepithelial lesions
Intraepithelial carcinoma
Bowen's Disease
Carcinoma in situ
vulvar atypia
Erythroplasia Queyrat
dysplasia bowenoid
Vulvar intraepithelial neoplasia
The beginnings of vulvar pathology …

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2006 Classification of dermatologic vulvar disease ISSVD
Spongiotic pattern
a)atopic dermatitis
b) Allergic contact dermatitis
c) Irritant contact dermatitis
Acantotic Pattern (previous benign squamous-cell)
a) Psoriasi
b) Lichen simplex cronicus
Lichenoid Pattern
a) Lichen sclerosus
b) Lchen planus
Sclerotic Pattern
a) Lichen sclerosus
b) Post-radiotherapy
Vescicous- bullous Pattern
a) Pemfigoidus
b) Linear IgA Disease
Acantolitic Pattern
a) Hailey-Hailey Disease
b) Darier Disease
c) Papular genitocrural acantholysis
Granulomatous Pattern Vasculopathic Pattern
a) Chron Disease a) Afthous ulcer
b) Melkenson-Rosenthal b) Bechet Disease
c) Plasma cell vulvitis

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International Society for the Study of
Vulvar Disease (ISSVD)
1970 The founding of ISSVD helped to establish an
ongoing dialog between representatives of the different
disciplines in an attempt to arrive at an accepted
nomenclature for diagnostic and therapeutic procedures
- - - > multidisciplinary approach
The first task was the systematic organization of the
terminology of "white lesions"

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Step 1 Define the lesion by choosing 1 or more of the following nouns
(Blister – Bulla – Cyst – Edema – erosion – Excoriation – Fissure –
Lesion – Macule – nodule – Papule – Plaque – Patch – Pustule –
Rash – Ulcer – Vesicle)
Step 2 Define color, margination , surface, configuration
Step 3 Formulate a list of differential diagnoses
(place an unrecognized vulvar disease into 1 of the 8 disease groups)
Step 4 Reduce the number of diagnoses in the list of differential diagnoses
(reading the brief sections on clinical morphology)
Step 5 Confirm a clinical diagnosis
(laboratory testing, biopsy )
DEFINITIONS, DESCRIPTION, AND DIAGNOSIS OF VULVAR LESIONS

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2011 ISSVD Clinical Classification of Vulvar Dermatological Disorders

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Complex classification
Based on both the pathophysiology and the clinical
manifestation of the disease
High average experience and knowledge of vulvar
pathology
Does not include colposcopic pattern

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The first doctor who deals with vulvar lesions
is often the gynecologist colposcopist
- Expert in diseases of the
female genital tract
- Colposcopic knowledge
is not fully comparable to
the vulvar pathology

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Terminology for colposcopists and not expert clinicians
Introduce basic and normal finding
Educate and assist not expert clinicans in the diagnosis
Include colposcopic pattern

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Normality and anormality coexist

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BASIC DEFINITION
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Urethra
Skene duct openings
Pubis
Labia majora, l. minora
Vestibule
Vestibular duct openings
Bartholin duct openings
Hymen
Fourchette
Perineum
Anus
Anal squamocolumnar junction (dentate line)
Vestibolo
Imene

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BASIC DEFINITION
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•
Composition:
Squamous epithelium: hairy/nonhairy, mucosa
•Clitoris
•Prepuce
•Frenulum

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NORMAL FINDINGS
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Micropapillomatosis

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NORMAL FINDINGS
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sebaceous glands (Fordyce spots)

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NORMAL FINDINGS
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Vestibular redness

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ABNORMAL FINDINGS
Lesion type
• Macule
• Patch
• Papule
• Plaque
• Nodule
• Cyst
• Vesicle
• Bulla
• Pustule

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Definition sof prymary lesion types
MACULE Small (<1.5 cm) area of color change, no elevation and no
substance on palpation
PATCH Large (>1.5 cm) area of color change, no elevation and no
substance on palpation
PAPULE Small (<1.5 cm) elevated and palpable lesion
PLAQUE Large(>1.5cm) elevated, palpable, and flap topped lesion
NODULE A large papule (>1.5 cm) often hemisherical or poorly
marginated; may be located on surface, within, or below the
skin; nodules may be cystic or solid
VESICLE Small fluid-filled lister; (<0.5 cm) the fluid is clear (blister: a
compartmentalized, fluid-filled elevation of the skin or mucosa)
BULLA A large fluid-filled blister; the fluid is clear (> 0.5 cm)
PUSTULE Pus-filled blister; the fluid is white or yellow
Miscellanee Traumi – malformazioni

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Each prymary lesion must be characterized for
- Size
- Location
- Color
- Secondary morphology
ABNORMAL FINDINGS

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ABNORMAL FINDINGS
Secondary morphology

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SUSPICION OF MALIGNANCY
Ulceration
Necrosis
Bleeding
Exophytic lesion
Hyperkeratosis
With or without white, gray, red, or brown discoloration

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ABNORMAL COLPOSCOPIC
Other magnification FINDINGS
• Acetowhite epithelium
• Punctation
• Atypical vessels
• Surface irregularities
Colposcopy of the vulva after the application of acetic acid is
discouraged as a routine examination

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Normal findings in vulvar examination and vulvoscopy
van Beurden M: BJOG 104:320;1997
40 Healthy women without vulvar symtomps
Median age 37.8 (range 21 -56)
In all women the vestibule resulted positive after application of acetic
acid to 5%

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ABNORMAL COLPOSCOPIC
Other magnification FINDINGS
33% Micropapillomatosis

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ABNORMAL COLPOSCOPIC
Other magnification FINDINGS
It can be useful
Evaluation of suspicious lesions
- VIN
- Vulvar cancer in early stage
- Warts
Better delineate the margins of the lesion
Identify the best site for biopsy

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ABNORMAL COLPOSCOPIC
Other magnification FINDINGS

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Vulvar diseases

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Lichen sclerosus
Common inflammatory dermatoses that affect the vulva
• F:M = 10 : 1 Wallace (1971): 1/300 - 1/10001
• Two peaks of presentation in women prepuberal girls and post
menopausal women
Aetiology:
Unknown
Multifactorial - genetic
- autoimmune
- environmental factors
NOTE: Often associated with autoimmune conditions, e.g. thyroid disease,
vitiligo, etc.
Familial cases have been reported

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Histology
Epidermis
• Epithelial/mucosal thinning
• Loss of epithelial digitations
Dermis
• Ialinic degeneration
• Oedema and inflammatory
linfoplasmacellular strip
between normal intermediate
and deep dermis

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Clinical Features
PHYSICAL EXAMINATION
•Ivory white papules or confluent plaques
•Cellophane-like sheen to surface
•Patchy or generalized - anywhere
• vulva, perineum, perianal
•Disease is not in the vagina
Secondary changes
- scratches, purpura, erosion
- crusting, thickening (lichenification)
- scarring with loss of normal
architecture; fusion of labia minora
-phimosis
-introital stenosis

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Symptoms
LS has a tremendous impact on the quality of life
by interfering with function and self image
Most common
- pruritus
- can be severe, intolerable
Can have soreness and burning
Often asymptomatic
Scratching results in open areas causing dysuria, pain,
dyspareunia, etc.

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• LS associated with vulvar squamous cell carcinoma
• 5% risk – possibly overestimated
• LS increases the 246-300x the relative risk of SCC
• Extra genital lesions not associate with malignancy
• 60% of vulvar SCC develop in a background of LS
• Incidence of vulvar cancer rising in the last years
• Weaker association with:
• Melanoma (Friedman RJ et al, 1984)
• Basal cell carcinoma (Meyrick Thomas RH et al, 1985)
• Verrucous carcinoma (Brisgotti M et al, 1989)
LS and malignancy
Renaud-Vilmer C, Cavelier-Balloy, B, Porcher R. Vulvar Lichen Sclerosus: Effect of Long-term Topical
Application of a Potent Steroid on the Course of the Disease. Arch Dermatol, Vol 140, June 2004
MacLean AB; Jones RW, Scurry J, Neill S. Vulvar Cancer and the Need for Awareness of
Precursor Lesions. J Low Genit Tract Dis. 2009 Apr;13(2):115-7

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LS and malignancy
Differentiated VIN
Usual type VIN
VULVAR CANCER
HPV
Lichen sclerosus
Walkden V, Chia Y, Wojnarowska F. The association of squamous cell carcinoma and lichen
sclerosus; implications for follow up. J Obstet Gynecol 1997; 17: 551–3
Vilmer C, Cavelier-Balloy B, Nogues C et al. Analysis of alterations adjacent to invasive vulvar
cancer and their relationship with the associated carcinoma: a study of 67 cases. Eur J Gynecol
60%
40%

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SCC occurs in 3-6 %
It is not a pre-malignant
lesion but increases
risk of carcinoma
Chronically scarring,
inflammatory dermatosis,
may act as and initiator
or promoter of
cancerogenesis

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Vulvar pigmented lesions
Naevi
Melanosis
Angiokeratoma
Melanoma

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Naevi

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Naevi

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Cortesia, Sezione Dermatologia
Università di Ferrara

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Physiologic and
post inflammatory hyperpigmentation

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Melanosis
Definition: benign pigmented lesion of the mucous membranes (oral,
genital) characterized by:
- Hyperpigmentation basal keratinocytes
- Melanocytes normal or only mildly elevated
- Presence of melanophages in the dermis

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Typical features
- symmetry
- smooth edges
- light brown
- size <6 mm
- often multiple
Melanosis

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In doubtful cases it may be useful dermoscopy
(microscopy epiluminescence)
Melanosis

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Cortesia, Sezione Dermatologia
Università di Ferrara

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Cortesia, Sezione Dermatologia
Università di Ferrara
Melanoma

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Vulvar pigmented lesions
• Diagnosis can be diffucult
• Dermoscopy is feasible and useful as
diagnostic tool
• Biopsy is mandatory in case of doubts

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To increase the knowledge of vulvar pathology
To Improve the diagnosis and treatment
To Standardize terminology
TAKE HOME MESSAGE
ISSVD e IFCPC

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Vaginal Intraepithelial Neoplasia
(VAIN)

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VAGINAL CANCER
 rare entity (1-2 % genital neoplasia)
 natural history not well known
 evolutive potential of VAIN not well
understood

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VAIN natural history
 23 cases
 3 yrs minim. follow up
 progression 9%
 persistence 13%
 regression 78%
Aho et al., 1991

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VaIN HETEROGENEITY
RELATED TO
 localizzation
 VaIN grade
 association with other
lower genital tract neoplasia

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VaIN localization
 third superior (cervical neoplasia)
 third inferior (vulvar neoplasia)
 multifocal (HPV infection)

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VaIN grade
 low grade
 high grade

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Low grade VaIN
 usually HPV related
 focal lesion (flat condyloma)
 multifocal
 CIN associated
 associated with vulvar warts
 associated with vaginitis

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TRETAMENT OF low grade VaIN:
General principles
 uncertain or low neoplastic potential
 therapy in relation to the underlying
disease
 focal lesions can be approached by
laser vaporization

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TREATMENTO OF LOW GRADE VaIN
Multifocal and diffuse
 treatment of the associated condition
 colposcopic follow up
 laservaporization
 chemotherapy (topical 5-FU)?

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TREATMENT OF HIGH GRADE VaIN
heterogeneity
 primary high grade VaIN
 associated with cervical neoplasia
 after hysterectomy
 associated to vulvar neoplasia

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TREATMENT OF HIGH GRADE VaIN
Primary VaIN
 laservaporization
 surgical excision (cold knife or laser)
 local chemotherapy (5-FU topical)?
 radiotheraphy

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CONCLUSIONS
 intraepithelial vaginal neoplasias rare
 VAIN heterogeneous entity
 ?therapy? Of low grade VaIN
 high grade VaIN can be approached by laser or
cold knife surgery
 occult invasive vaginal cancer is frequently
found in surgical specimens of colpectomy for
high grade VaIN after hysterectomy for CIN3

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