Vulvar Dystrophies

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Vulvar Dystrophies
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ICVSS Classification (1985)

• Lichen Sclerosis
• Squamous Hyperplasia
• Benign Dermatoses
• Intraepithelial Atypia
• VIN I Mild Dysplasia
• VIN II Moderate Dysplasia
• VIN III Severe Dysplasia CIS

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Lichen Sclerosis

• Gross
• Whitish change of the vulva
• Decreased subQ fat (atrophic vulva)
• Small/Absent labia minora, Thin Majora
• Occasional Phimosis of Prepuce
• Pale, shiny, crinkled surface pattern, w/
  fissures and excoriation (cigarette paper)
• Symmetrical, often extending to perineal or
  perianal area

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LS. Vulva has diffuse, white, parchmentlike
appearance.
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LS in 10 y.o. w/ excoriation bleeding above
clitoris.
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Lichen Sclerosus

• Histologic
• Thinned epithelium
• loss or blunting of rete ridges (pegs)
• Thickening (hyperkeratosis) of surface layers
• Inflammation infiltrate present
• Underlying collagenization

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Lichen Sclerosus w/ hyperkeratosis
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Lichen Sclerosus

• Symptoms
• Initially pruritis
• Shrinkage of vuvlar skin
• Introital stenosis
• Fissure formation of posterior fourchette - leads
  to painful intercourse
• Note Senile Atrophy and Atrophic Vulvitis
  are typically Lichen Sclerosus

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Lichen Sclerosus

• Clinical
• Occurs mostly in post-menapause
• If pre-menausal, non-symptomatic typically
• NOT premalignant
• Tends to be multi-focal and to recur locally
• May lead to carcinoma if scratched excessively
  itch-scratch-lichen sclerosus hypothesis (Scurry,
  IJGC, March 1999)

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Lichen Sclerosus

• Increased chance of distant CA with LS.
• Hart Study (Obstet Gynecol, 1975)
• 107 pts w/ LS followed for 12 yrs
• 1 developed vulvar CA
• 5 had vulvar CA at time of LS diagnosis
• 12 developed other CA Cervix, breast, colon,
  ovary, endometrium

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Lichen Sclerosus

• Treatment
• 2 Testosterone Proprionate cream
• BID x 3 wks Qd x 3 wks QOD/biweekly maintenance
• Long term use causes masculinzation due to
  systemic absorption clitoral hyperplasia,
  increased hair growth. Local Progesterone cream
  can be tried if s/e are excessive.

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Lichen Schlerosus

• Treatment
• Cortiocosteroid Clobetasol Propionate (0.5)
• BID x 12 wks results in 80-100 improvement
  (Bracco, J Reprod Med, 1993)
• Reduces hyperkeratosis, thickens epithelium
• More effective than testosterone, especially in
  long term (Bornstein, AJOG, Jan 1998)
• Long term s/e Atrophy, telangiectasia, striae of
  skin
• Still unclear as to a good maintenance program
  Clobetasol x 24wks then Testosterone x 24wks was
  shown to be less effective than Clobetasol alone
  placebo (Cattaneo, OGS, June 1996)

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Lichen Sclerosus

• Treatment Puritis
• Fluorinated Corticosteroids
• 0.025-0.1 Triamcinolone Acetonide (Aristocrot,
  Kenalog), Fluorocinolone Acetonide (Synalar),
  0.01 Betamethasone valerate (Valisone)
• BID x 2 wks then taper off
• long term use vulvar atrophy contraction

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Lichen Sclerosus

• Treatment Puritis
• Long term Nonfluorinated corticosteroid
• 1.0 Hydrocortisone
• 3 Doaks Tar Hydrocortisone in severe cases
• Occasionally sufficient for initial therapy

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Lichen Sclerosus

• Therapy Contractures
• Woodruff describes surgical technique to repair
  posterior fourchette

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Squamous Hyperplasias

• Typical Hyperplastic Dystrophy
• Atypical Hyperplastic Dystrophy
• Mixed Hyperplastic Dystrophy

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Squamous HyperplasiasTypical Hyperplastic
Dystrophy

• Character Hyperkeratosis, elongation and
  blunting of rete pegs (acanthosis), NORMAL
  maturation of proliferative epithelium
• Underlying inflammatory infilitrate
• Can occur in any age group
• Once confirmed by biopsy, treated symptomatically
  via fluorinated hydrocortisone vs. pruritus

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SCH w/ diffuse thickening and red appearance of
vulva
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SCH. Vulva has diffuse, thick and white
appearance
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Squamous HyperplasiasAtypical Hyperplastic
Dystrophy

• Proliferation of the basal and parabasal cells w/
  occasional mitosis (more than normal mitosis)
• Atypical maturation (keratinization) at rete tips
  - most ominous change
• Keratinized cells in basal layer forming pearls
• Pre-malignant lesion

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Squamous HyperplasiasAtypical Hyperplastic
Dystrophy

• Wide local excision or vulvectomy
• Careful follow-up every 6-9 months to watch for
  vulvar cancer in adjacent skin
• Usually in patients older than 50.

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Squamous HyperplasiasMixed Hyperplastic
Dystrophy

• Combination of Lichen Sclerosus and Hyperplasia
• If biopsy shows atypical changes, wide excision.
  Otherwise, symptomatic treatment.

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LS w/ epithelial hyperplasia. Several
excoriations can be seen
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LS w/ epithelial hyperplasia. Previous
vulvectomy w/ recurrence
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Pagets Disease

• Rare intraepithealial disorder
• Resemble Pagets disease of the breast
• Characterized by large pale cells (Pagets Cells)
  w/ vacuolated cytoplasm
• Occur in nests and infiltrate upward through
  epithelium
• Histologic abnormalities of apocrine glands.

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Pagets Disease of the Vulva
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Pagets Disease

• More common in Caucasians gt65 y.o.
• Not associated with future carcinoma at site, but
  disease will extend locally
• Associated with increased probability of invasive
  carcinoma at remote sites
• SQ CA cervix, vulva
• Adeno CA of sweat glands of vulva, or Batholins
  Glands
• GI, Breast, Urethral, Basil Cell CA.

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Pagets Disease

• Lee Study (Cancer, 1977) 75 cases of Pagets
• 22 underlying CA of adenexal skin structures
• 9 CIS
• 29 CA at distant sites

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Pagets Disease

• Fanning (AJOG, Jan 1999) 100 cases
• 26 non-vulvar CA
• 4 AdenoCA of vulva
• No SQCA of vulva, No CIS
• 7 year f/u 34 recurrence
• 31 for radical vulvectomy
• 20 for radical hemivulvectomy
• 43 for wide excision

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Pagets Disease

• Treatment
• Excision must include underlying dermis, since
  even non-invasive Pagets can occupy underlying
  adnexal structures

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Diagnosis of Atypias

• Sx Irritation and Itching
• Whitish change due to thickened keratin layer
  (old term leukoplakia no longer used, since
  denotes pre-malignancy, but most white lesions
  are not)

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Diagnosis

• LS diffuse white change, thinning of skin,
  contractures scarring
• Squamous Hyperplasias Whitish lesions, but
  thickened skin, more focal or multi-focal
• Pagets Reddish eczematoid appearance
• All lesions must be biopsied.

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Diagnostic Methods

• Cytology Not as useful with the vulva due to
  thick keratinized epithelium, does not shed cells
  well
• May be useful if ulceration

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Diagnostic Methods

• Toluidine blue test Stains nuclei in superficial
  epithelium (keratin layer), which normally does
  not contain nuclei
• 1 touludine blue, let dry 1 minute
• 1 acetic acid applied then removed w/ cotton
  swab
• 2/3 of vulvar atypias retain dye, but ulcerations
  and fissures also retain dye

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Diagnostic Methods

• Colposcopy of vulva
• difficult since vascular pattern and tissue
  changes seen in cervical abnormalities are not
  seen in the vulva.
• Increased magnification may be useful for
  following previous excisions, but is not useful
  for routine screening.

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Diagnostic Methods

• Biopsy
• Keyes dermal punch
• 4-5 mm diameter
• If larger biopsy is required, a scalpel or
  cervical punch biopsy can be used

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Vaginitis

• C. albicans
• Gardnerella
• Trichomonas

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Vaginitis

• Vaginal discharge from vaginitis is 1 presenting
  gynecologic symptom
• Other presenting sx related to vaginitis
  dysuria, odor, vulvar burning and pruritis
• In middle-class, reproductive age women
• 50 Bacterial
• 25 Candidiasis
• 25 Trichomonas

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Vaginitis

• Nl pH 3.8-4.2, due to glycogen conversion to
  lactic acid by nl flora (lactobacilli, g rod)
• pH gt 5.0 bacterial or trichomonas, or
  post-coital

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Vaginitis

• Discharge
• Nl White, odorless
• Bacterial Grey-white, thin, foul odor, adherent
• Trichomonas Yellow-Grey, thin
• Candidiasis White, curdy

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Vaginitis

• Wet smear
• Candida Budding filaments, pseudohyphae
• Gardnerella Clue cells
• Trichomonas WBCs, protozoa

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Bacterial Vaginitis

• Sexually transmitted, 5-10 d incubation
• Gardnerella vaginalis g- bacillus
• 104/ml in asymptomatic women, 107 in symptomatic
  women
• Increase in anaerobic bacteria, decrease in
  lactobacilli in symptomatic women
• Risk factors IUD, nonwhite, prior pregnancy
• Increased risk of preterm birth in women with
  bacterial vaginitis

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Bacterial Vaginitis

• Fishy or Musty odor following release of
  aromatic amines
• whiff test - 10 KOH. Semen is alkaline, same
  effect (also positive in trichomonas)
• Clue Cells
• Vaginal epithelial cells w/ clusters of adherent
  bacteria to external surfaces
• in 2-50 of infected women
• Wet mount shows lack of lactobacilli

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Bacterial Vaginitis

• Diagnosis Criteria
• 1) Homongenous vaginal discharge
• 2) pH gt 4.7
• 3) positive whiff test
• 4) 20 epithelial cells are clue cells

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Bacterial Vaginitis

• Metronidazole (Flagyl)
• 500mg BID x 7 days
• Pfeifer (NEJM, 1979) 80/81 women treated
  responded
• 1g BID x 1 day
• Purdon (ObsGyn 1984) 75 response rate

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Bacterial Vaginitis

• Others
• Ampicillin less effective (66), and lactobacilli
  does not return as well
• Topical vaginal therapy with 2 percent
  clindamycin or 0.75 percent metronidazole gel has
  been shown to be as effective as oral
  metronidazole (Ferris, J Fam Practice, 1995)
• Despite being sexually transmitted, treatment of
  male partner has not been shown to be effective