1. Shelley Miksis, DNP, ARNP 01-21-10
Vulvar Conditions
Shelley Miksis, DNP, ARNP

2. Plan for today’s session
General considerations
Basis for differential
Evaluation History, PE, Dx procedures
Management
Focus on specific conditions VIN, vulvar CA, LS, LP, & hyperplasia

3. General Considerations
Wide array of benign, premalignant & malignant lesions
Eyes alone insufficient to tell benign from malignant
Biopsy needed for diagnosis and to identify neoplasia

4. General Considerations
Symptoms related to vulvar disorders include:
Pruritus
Vulvodynia
Superficial dyspareunia
Lesions
White, red, pigmented, raised, or ulcerated
Patient may be asymptomatic

5. General Considerations
Vulvar symptoms may be caused by:
Infections
Dermatologic disorders
Neoplastic vulvar disorders
Non-neoplastic vulvar disorders

6. Definition of terms Neoplasia= Formation of new tissue, neoplasm
Neoplasm = An abnormal new growth of tissue that grows by cellular proliferation more rapidly than normal, continues to grow after the stimuli that initiated the new growth cease, shows partial or complete lack of structural organization and functional coordination with the normal tissue, and usually forms a distinct mass of tissue which may be either benign or malignant.

7. Classification: Non-neoplastic
Non-neoplastic epithelial disorders of skin and mucosa
Lichen sclerosus
Squamous hyperplasia
Other dermatoses [ psoriasis, lichen simplex, lichen planus, dermatits, etc. ]

8. Classification: Neoplastic
1986 ISSVD Classification System for Vulvar Intraepithelial Neoplasia (VIN)
VIN I mild dysplasia
VIN II moderate dysplasia
VIN III severe dysplasia and
carcinoma in situ or CIS

9. Problems with Old VIN Classification
Natural history of VIN 1, 2, and 3 does not progress on a continuum.
VIN 1 is not a precursor to cancer
VIN 1 has not shown to be a reproducible or reliable diagnosis
No reliable distinction between VIN 2 and 3

10. 2004 VIN Classification Changes
Classification based on morphologic criteria
VIN I designation has been eliminated
Low malignant potential
Not a precursor to VIN 2 or 3
Does not require treatment
Term VIN is now limited to histologically high grade squamous lesions (formerly VIN 2 and 3)
Significant potential for progression to invasive cancer.
Requires treatment

11. The natural history of VIN 1, 2, and 3 does not progress as a continuum like CIN, the new system thus eliminated VIN 1 and combined VIN 2 and 3. VIN 1 has not been shown to be a reproducible or reliable diagnosis, and there is likewise no reliable diagnostic distinction between VIN 2 and 3.

12. Classification: Neoplastic
2004 ISSVD Classification System for VIN
a. VIN, usual type (r/t high risk HPV)
Warty type
Basaloid type
Mixed (warty, basaloid) type
b. VIN, differentiated type (not r/t HPV)

13. Classification: Neoplastic
VIN, usual type – most common
Basaloid and warty subtypes based on morphologic and histologic features
Basaloid – thickened epithelium with relatively flat, smooth surface
Warty – undulating or spiking surface, giving condylomatous appearance

14. Approach to differential
Based on morphology of lesion, not symptoms
White lesions
Red lesions
Dark or pigmented lesions
Ulcerative or erosive lesions
Solid and cystic tumors

15. Differential: Key to diagnoses listed
If a disease or condition  regular font
If an infection  Italics
If malignant or pre-malignant  bold

16. White lesions Condyloma acuminate / genital warts Lichen sclerosus
Post-inflammatory hypopigmentation
Squamous cell hyperplasia
VIN
Vitiligo

17. Lichen sclerosus

18. White plaques of VIN

19. Condyloma acuminata

20. Red Lesions Allergic or contact/irritant dermatitis
Cutaneous candidiasis
Lichen planus
Paget’s Disease
Psoriasis
VIN

21. Red macular lesion of VIN

22. Paget’s disease

23. Vulvar psoriasis

24. Allergic contact dermatitis: Neck

25. Dark lesions Acanthosis negricans Basal cell carcinoma Lentigo
Melanoma
Nevi
Post-inflammatory hyperpigmentation
Seborrheic keratosis
VIN

26. Acanthosis Nigricans

27. Brown macular lesion of VIN

28. Superficial spreading melanoma

29. Dysplasic nevus
Note: the dysplastic nevus has irregular borders and multiple colors.

30. Lentigo

31. Lentigo maligna melanoma

32. Seborrheic keratosis

33. Ulcerative lesions Basal cell carcinoma Erosive lichen planus
Genital herpes
Primary syphilis
Squamous cell carcinoma

34. Solid & Cystic Tumors Small lesions (usually < 1 cm in diameter)
Acrochordons (skin tags)
Epidermal cysts
Hidradenitis suppurativa

35. Solid & Cystic Tumors Large lesions Bartholin’s Cyst
Bartholin’s Abscess
Verrucous carcinoma

36. Bartholin’s gland abscess

37. Evaluation Consider both age and immune status
Higher risk of malignancy if post-
menopause
Immune compromised
- Increased risk of VIN & vulvar cancer
- Exaggerated presentations of
common infections

38. Evaluation: History 1. Onset - how long lesion has been present?
2. Character - initial appearance?
current appearance?
3. Location - where on genitals? similar lesions elsewhere?
4. Timing - come and go? always there?
5. Course - staying the same? getting worse?

39. Evaluation: History
6. Self treatment and outcome What’s been tried? ( herbal, OTC, Rx meds ) Response to each thing tried? ( better, worse, no change )
7. Aggravating & alleviating factors What makes it worse? What makes it better?

40. Evaluation: History Itching? 8. Associated symptoms focused on lesion
Pain?
Burning or Stinging?
Feeling of rawness?
Dampness?
Bleeding?

41. If itching, ask… How intense is the itching?
Does itching awaken you from sleep?
Amount of scratching in response to itching?

42. Evaluation: History Additional associated symptoms re:
Vaginal infections
STIs
Cervical cancer
Derm conditions
Low estrogren state
Vulvar hygiene - See Outline, page 13
Dyspareunia

43. Evaluation: History Related PMH Vulvar conditions Cervical dysplasia
HPV status
Cervical, uterine, or ovarian cancer
Allergies; asthma; skin problems
Lowered immune status
HIV status

44. Evaluation: History Previous occurrences
Related FH: Diabetes, skin problems
Patient profile
-- Stress
-- Tobacco use
Impact on ADLs / quality of life

45. Evaluation: PE Not all vulvar conditions are symptomatic
Careful inspection of external genitalia
If find an asymptomatic lesion, then SA

46. Evaluation: PE Inspect skin non-genital areas, esp skin folds
Inspect mucous membranes
Lymphadenopathy – inguinal
External genitalia and vulva
Speculum exam, if indicated

47. PE: more detail Inspection of external genitalia and vulva
 Good light essential
 Spread hair/labia/folds to inspect all aspects

48. PE: Lesion characteristics
Type of lesion
Size & shape of individual lesions
Solitary –or-- multiple & pattern
Color
Texture & if tender
Secondary changes– crusts, etc.

49. Diagnostic procedures
Macroscopic
- Magnifying lens
- Colposcopy
Microscopic
- wet prep
- vulvar cytology
3. Biopsy

50. Biopsy if suspicious for malignancy
asymmetry
irregular border
variable color
bleeds
rapidly changing
does not heal
slight ulceration in raised lesion

51. Also biopsy if… Diagnosis is not clear
Lesion does not resolve w/ therapy
Patient concerned & wants biopsy

52. Biopsies: Types
Shave
Punch
Excisional
Incisional

53. Management: General Good vulvar hygiene Soaks Corticosteroids
Estrogen (topical)
Pt education
Follow-up

54. Now for specific conditions…

55. VIN Vulva, vagina, cervix, and anus share same embryonic origin
Oncogenic stimulus (e.g.HPV) neoplasia
Neoplasia influenced by host reaction
VIN, usual type and differentiated  have malignant potential

56. VIN: Risk Factors
intraepithelial neoplasia in other lower genital tract sites
HPV infection
immunocompromised
smoking
chronic vulvar irritation
lighter skin pigmentation

57. Old VIN I – Now Condyloma/HPV Effect
Well localized and delineated
Flat or slightly elevated
White and rough
Less common red-brown

58. VIN, usual type (warty, basaloid, and mixed)
Most common type of VIN (90-95%)
Precursor lesion to HPV-associated invasive squamous cell carcinoma (SCC)
HPV associated (HPV types 16, 18, 31)
Presents in younger, premenopausal women

59. PE findings: VIN, usual type
Multifocal lesions
Well localized and delineated
Lesions most often in interlabial grooves, posterior fourchette, & perineum
Slightly elevated, white-gray, rough
Less common – red-brown color or red-white patches

60. VIN, usual type white-gray lesion

61. VIN, usual type red lesion

62. VIN, usual type brown lesion

63. VIN, differentiated Less common type of VIN (6-10% of cases)
Frequently associated with SCC, LS, Squamous hyperplasia
Mainly postmenopausal women
Not related to HPV

64. PE findings: VIN, differentiated
Commonly encountered in background of lichen sclerosis
Unifocal lesions
Erosive or ulcerative areas
Hyperpigmented, fixed, or indurated lesions
Warty papule
Hyperkeratotic plaque

65. White plaques of VIN

66. VIN

67. VIN: Diagnosis Early dx depends on regular vulvar exams
High index of suspicion
If suspect, biopsy (via colposcopy best)
Refer confirmed VIN to MD

68. VIN: Treatment Wide local excision Laser vaporization
Skinning vulvectomy (w/ or w/out graft)
Immunomodulators
*Agents that enhance or induce a strong cell-mediated immune response likely hold the greatest promise not only for control of HPV-related disease, but also for reduction of future recurrences

69. Vulvar Cancer 4th most common gyn cancer
Bimodal age distribution – represents two distinct etiologies
Young women – related to HPV (60% of vulvar CAs)
Older women – not related to HPV (chronic inflammatory or auto-immune process)
Most ( > 90%) are squamous cell CAs
Risk factors same as VIN risk factors, plus if PMH of VIN
Many women are asymptomatic

70. Vulvar Cancer: Symptoms
Most common  Pruritus
Less common, when more advanced
-- vulvar bleeding or discharge
-- dysuria
-- enlarged lymph node in groin

71. Vulvar Cancer: PE Findings
Most often
 unifocal vulvar plaque, ulcer, or
mass (fleshy, nodule, or warty)
on labia majora

72. Vulvar Cancer

73. SCC and LS

74. Vulvar Cancer: Diagnosis
Histological evaluation essential diagnosis depth of involvement  biopsy center of lesion
If suspect or diagnose vulvar cancer, must refer to an MD

75. Lichen Sclerosus
Chronic, progressive, inflammatory skin condition found most often in the anogenital region.
Does not occur in the vagina
Accounts for ~70% of non-neoplastic vulvar lesions
Occurs most in post-menopausal women, although not exclusively

76. Lichen Sclerosus: Symptoms
 PRURITIS is HALLMARK of LS
Intensity may awaken
Other symptoms:
Rectal itching, fissures, bleeding, painful defecation
Dyspareunia
Decreased sexual sensation, anorgasmia
Dysuria, difficulty voiding
*May be asymptomatic – 1/3 of patients

77. LS: PE findings
Classic LS = thin, white, wrinkled skin on labia minora and/or labia majora. “Parchment-like”
“Keyhole” pattern
Fissures in labial folds, around clitoris or anus
Excoriations and lichenification r/t scratching
Telangiectasia / hematoma / ecchymoses
Changes in vulvar architecture

78. LS: Changes in vulvar architecture
None early in course of LS
Labia majora/minora become less distinct.
- Adhesion of labia minora to majora
Clitoris covered under fused prepuce.
- Edema or agglutination of prepuce and
frenulum “bury clitoris”
Stenosis or constriction of introitus

79. Lichen sclerosus

80. Lichen Sclerosus

81. Lichen sclerosus White appearance from: hyperkeratosis
loss of pigmentation
relatively less vascularity

82. LS: Keyhole pattern
Perianal LS

83. LS: Loss of architecture
Asymptomatic in 1/3 of
patients.
Can progress to
scarring and loss of
vulvar architecture

84. Lichen Sclerosus

85. When suspect LS, differential includes:
Lichen planus
Squamous cell hyperplasia (usually lichen simplex chronicus)
Vitiligo
Psoriasis
Candidiasis

86. When suspect LS… MUST rule out VIN and vulvar cancer
before initiating treatment.
Women w/ vulvar LS have increased risk for invasive squamous cell cancer.

87. LS: Biopsy Biopsy to confirm diagnosis
 3-4 mm punch  Specimen from advancing margin
Biopsy to identify VIN or vulvar CA
 Biopsy center to ensure sample most severe pathology

88. LS: Management Initiate treatment asap, even if asymptomatic
Wet dressings or soaks x min w/ Burrow’s sln
Corticosteroids  relief of pruritus and resolution of hyperkeratosis, fissures, and ecchymoses.
-- Will not reverse atrophy, whiteness or scarring.
Rx: Clobetasol or halobetasol propionate 0.05%
ointment BID x 4 weeks, then qhs x 4 weeks,
then 1-3 x per week for maintenance.
Directions: Spread sparingly to cover affected area with thin film

89. LS: Management Evaluate for possible associated problems
Autoimmune disorders, e.g., alopecia areata, vitiligo, thyroid disease, and pernicious anemia (21% - most commonly thyroid disorder)
Premature menopause
Infection

90. LS Management: Patient Education
Chronic, progressive condition; ? cause
Symptom relief and treatment options
Lesions do not always disappear w/ tx
Do NOT stop treatment when itching stops!
Continued topical therapy can slow progression
Encourage sufficient sleep, diet, and exercise
Encourage stress reduction techniques
Support group available, if interested
Regular self and clinician evaluation essential

91. LS Management: Follow-up
On-going evaluation is essential
-- Visits every 1-3 months until stable
-- Every 6 months while stable
Biopsy progressive, recurrent, persistent, or suspicious lesions – risk of SCC.
Refer to MD, if not responsive to therapy

92. Lichen Planus
An inflammatory autoimmune skin disorder which may affect only the vagina, vulva or may occur elsewhere on skin; also nails and mucous membranes
Vulvar LP is uncommon
Peak incidence, women yrs old

93. Lichen Planus: Symptoms
Irritating vaginal discharge and/or vulvar soreness, thought to be yeast infection
Intense pruritus
Burning
Dyspareunia
Post-coital bleeding

94. Lichen Planus: 3 types Papulosquamous LP:
small, violaceous, intensely pruritic papules on keratinzed skin.
Papules are poorly demarcated, pink, and opaque.
Associated w/ “milky striae” on inner aspects of the labia.

95. Lichen Planus: 3 types 2. Hypertrophic LP:
hyperkeratotic, rough lesions on perineum and perianal area.
May appear similar to squamous cell CA.

96. Hypertrophic lichen planus

97. Lichen Planus: 3 types 3. Erosive LP: Most common variant of LP
Involves vagina 70% of the time
Violaceous erosions that look like glassy, reticulated, white papules and plaques.
White striae along lesion margins.
Progression leads to extensive erosion and ulceration and destruction of vulvar architecture.

98. Erosive Lichen Planus

99. Erosive Lichen Planus

100. Lichen Planus

101. Lichen Planus

102. Lichen Planus

103. Lichen Planus

104. Lichen Planus If suspect lichen planus refer to MD
Hard to diagnosis! Difficult to treat!
If suspect lichen planus refer to MD

105. Squamous cell hyperplasia
Most squamous cell hyperplasia is lichen simplex chronicus
Occurs in all ages
Thickened skin (lichenification) is result of scratching or rubbing
 Squamous cell hyperplasia may coexist with LS

106. Lichen Simplex Chronicus

107. Lichen Simplex Chronicus

108. Squamous cell hyperplasia
Diagnosis of exclusion. Need to rule out—
Lichen sclerosis
Psoriasis
Lichen planus
Eczema
HPV
Candidiasis

109. Squamous cell hyperplasia
Consider possibility of malignancy (VIN,
vulvar CA) before starting treatment.
Goal of treatment is to—
break “itch-scratch” cycle

110. Squamous cell hyperplasia: Treatment
Mild symptoms – use low to medium potency corticosteroid ointments
Hydrocortisone 1 –or- 2.5 % Triamcinolone 0.1 %
Rx: Apply twice daily for 2-4 weeks, then x 2 /week.
Continue therapy at min frequency to control pruritus.
More than mild symptoms – use high potency corticosteroid ointment
-- Clobetasol propionate 0.05% ointmt each night x 30
days, then re-evaluate [usually 2-3 months, then taper]

111. Squamous cell hyperplasia: Treatment
If night time itching  scratching,
Rx sedating antihistamine (e.g., hydroxizine) in pediatric dosage ( 10 mg at hs)

112. Consulting & Referring
Vulvar biopsy
Consult if uncertain if vulvar biopsy indicated
Refer if not skilled in vulvar biopsy techniques
VIN
Consult if suspect VIN
Refer if VIN diagnosed

113. Consulting & Referring
Management
Whenever unfamiliar with indicated meds Consult with NP/PA specialists in derm, pharmacist, or MD to ensure appropriate med, dose, route, and timing.
Refer if lesion persists despite treatment
Refer whenever surgery or laser treatment is indicated (e.g., for VIN)