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Vulvar Dystrophies

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Vulva has diffuse, thick and white appearance LS w/ epithelial hyperplasia. Several excoriations can be seen LS w/ epithelial hyperplasia. – PowerPoint PPT presentation

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Title: Vulvar Dystrophies


1
Vulvar Dystrophies
2
ICVSS Classification (1985)
  • Lichen Sclerosis
  • Squamous Hyperplasia
  • Benign Dermatoses
  • Intraepithelial Atypia
  • VIN I Mild Dysplasia
  • VIN II Moderate Dysplasia
  • VIN III Severe Dysplasia CIS

3
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

4
LS. Vulva has diffuse, white, parchmentlike
appearance.
5
LS in 10 y.o. w/ excoriation bleeding above
clitoris.
6
Lichen Sclerosus
  • Histologic
  • Thinned epithelium
  • loss or blunting of rete ridges (pegs)
  • Thickening (hyperkeratosis) of surface layers
  • Inflammation infiltrate present
  • Underlying collagenization

7
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8
Lichen Sclerosus w/ hyperkeratosis
9
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

10
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)

11
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

12
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.

13
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)

14
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

15
Lichen Sclerosus
  • Treatment Puritis
  • Long term Nonfluorinated corticosteroid
  • 1.0 Hydrocortisone
  • 3 Doaks Tar Hydrocortisone in severe cases
  • Occasionally sufficient for initial therapy

16
Lichen Sclerosus
  • Therapy Contractures
  • Woodruff describes surgical technique to repair
    posterior fourchette

17
Squamous Hyperplasias
  • Typical Hyperplastic Dystrophy
  • Atypical Hyperplastic Dystrophy
  • Mixed Hyperplastic Dystrophy

18
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

19
SCH w/ diffuse thickening and red appearance of
vulva
20
SCH. Vulva has diffuse, thick and white
appearance
21
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

22
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23
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.

24
Squamous HyperplasiasMixed Hyperplastic
Dystrophy
  • Combination of Lichen Sclerosus and Hyperplasia
  • If biopsy shows atypical changes, wide excision.
    Otherwise, symptomatic treatment.

25
LS w/ epithelial hyperplasia. Several
excoriations can be seen
26
LS w/ epithelial hyperplasia. Previous
vulvectomy w/ recurrence
27
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.

28
Pagets Disease of the Vulva
29
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30
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31
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.

32
Pagets Disease
  • Lee Study (Cancer, 1977) 75 cases of Pagets
  • 22 underlying CA of adenexal skin structures
  • 9 CIS
  • 29 CA at distant sites

33
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

34
Pagets Disease
  • Treatment
  • Excision must include underlying dermis, since
    even non-invasive Pagets can occupy underlying
    adnexal structures

35
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)

36
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.

37
Diagnostic Methods
  • Cytology Not as useful with the vulva due to
    thick keratinized epithelium, does not shed cells
    well
  • May be useful if ulceration

38
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

39
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.

40
Diagnostic Methods
  • Biopsy
  • Keyes dermal punch
  • 4-5 mm diameter
  • If larger biopsy is required, a scalpel or
    cervical punch biopsy can be used

41
Vaginitis
  • C. albicans
  • Gardnerella
  • Trichomonas

42
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

43
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

44
Vaginitis
  • Discharge
  • Nl White, odorless
  • Bacterial Grey-white, thin, foul odor, adherent
  • Trichomonas Yellow-Grey, thin
  • Candidiasis White, curdy

45
Vaginitis
  • Wet smear
  • Candida Budding filaments, pseudohyphae
  • Gardnerella Clue cells
  • Trichomonas WBCs, protozoa

46
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

47
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

48
Bacterial Vaginitis
  • Diagnosis Criteria
  • 1) Homongenous vaginal discharge
  • 2) pH gt 4.7
  • 3) positive whiff test
  • 4) 20 epithelial cells are clue cells

49
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

50
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
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