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Vaginal and Vulvar Diseases:

 

I.  Vulvar Diseases can manifest in the following ways:

a.       Systemic diseases that affect the entire body can manifest on the vulva since it is part of the body à Crohn’s disease

b.       Because the a portion of the vulva is skin-like, dermatological diseases can also manifest on the vulva - Psoriasis (like that which appears on the elbow)

c.       Vulvar specific conditions = conditions that pertain specifically to the vulva such as tumors or neoplasms in any of the parts of the vulva

Inflammatory Conditions that are not infections:

a.       Nonneoplastic Epthelial Disorders (according to the International Society for the Study of Valvular Diseases - ISSVD)

                                                              i.      Lichen Sclerosis

1.      Gross, thin, white (due to the hyperkeratosis), parchment-like vulvar skin with  cigarette-like paper wrinkling

2.      Loss of anatomy = fusion of the hood over the clitoris, loss of delineation of the labia minora, etc = can make sexual intercourse impossible;

3.       Histologically will see thinning of the epithelium, hyperkeratosis, hematogenous dermal layer (appears glassy under the microscope), and a decrease in the Rini pegs of the epidermis

4.      Probably a hypermetabolic condition = appears atrophic (was once called Lichen sclerosus anatrophic condition)

5.      Very pruritic (one of the main reasons why women see their gynecologist)

6.      More common in elderly Caucasian women and the pediatric age group (not as severe in them); may be seen in adolescents

                                                            ii.      Squamous cell hyperplasia (Wire-brush ladies)

1.      Analogous to Lichen simplex chronicus on the body = uninterrupted itch/scratch cycle

2.       Thickened, coarsened skin due to uninterrupted itch/scratch cycle – women present with such an intense pruritic vulva that they take a wired brush to scratch = not good

3.      Hyperkeratosis and increase in the Rini pegs of the epidermis

 

 
iii. Tumor-like Lesions - Benign Neoplasms

1. Bartholin's Duct cyst/abscess

2. Epidermal inclusion cysts, mucinous cysts

3. Skin tags

4. Hemangioma

5. Fibroma

6. Lipoma

b.   Vulvar Intraepithelial Neoplasia (VIN) à

                                                               i.      Just one of several types of Intraepithelial Neoplasia (Cervial Intrapeithelial Neoplasia is CIN; Perianal Intraepithelial Neoplasia is PAIN which is probably painful; vaginal is VAIN)

                                                             ii.      Most are related to HPV since HPV likes squamous epithelium of the female genital tract i.e. common in the vulva, vagina, cervix and perianal region

                                                            iii.      They have not broken through the basement membrane and has no metastatic potential

                                                            iv.      Requires liberal biopsy *You have seen this terminology used several times now. Current gynecologists are trained to “biopsy liberally” as a lot of things present similarly histologically. 

                                                             v.      Classification

1.       Low grade vs. high grade (consists of 2 & 3, considered to be the most clinically significant of the three and treated similarly)

2.       Classification based on what is seen microscopically à

a.       VIN 1 (lower 1/3 of epithelium)

b.       VIN 2 (2/3 of epithelium)

c.       VIN 3 (entire epithelium)

                                                            vi.      Presentation is highly variable = single/multiple lesions, macular, popular, variable color, may itch

                                                           vii.      Histologically = increase in the Rini pegs, darkness due to maturation abnormality in which cells do not develop (cells are not uniform and appear small and dark).

                                                         viii.      Classic VIN à

1.       Recurs/persists in 7-22% of cases

2.       Occult invasion in 6-7% (up to 19% in one study series)

3.       Progression to invasion in 3-10% = greater with inadequate or no treatment therefore treat them

4.       Risks for subsequent invasion increases if >40yo (older) and/or immunocompromised

5.       2-7% of pts diagnosed prior with “vulvar dystrophy” (the old name given to non-neoplastic disorders) actually have VIN

6.       40% are multifocal

7.       Various clinical appearances = multiple red or white plaques, pigmented, polypoid, verruciform or flat

8.       Associated with both in situ and invasive cervix and vaginal lesions in 18-52% of the cases

                                                           ix.      HPV field effect = if someone presents with an HPV lesion in one place needs to be checked elsewhere

c.   Paget’s Disease of the Vulva = non-HPV-related type of Intraepithelial Neoplasia

                                                              i.      Itch is the most common presentation

                                                             ii.      Histologically looks similar to Paget’s disease of the breast yet the two are different

1.       Both result from some type of cell percolating up from subcutaneous tissues into the epithelium

2.       That of the breast is believed to be neoplastic cells so considered some type of breast cancer

3.       That of the vulva is believed to be an aberrant stem cell that is associated with invasive cancer in 25-30%

4.       Not always a vulvar cancer = can also be other cancers such as sweat gland adenocarcinoma. Or can signify that you have cancer of the GI tract.

 

                                                            iii.      Associated with ~25-30% of underlying in situ or invasive cancer (Paget’s disease has a 100% association)

                                                            iv.      Most common in Caucasian elderly women

                                                              v.      Multifocal

                                                           vi.      Extends beyond what you can see during examination = one of the reasons for its high recurrence

                                                           vii.      Histologically = big, pink cells that spread to the surface (melanoma also histologically spreads so need to distinguish with special stains)

d.  Vulvar cancer – less common than the other cancers

                                                               i.      More common in older women who do not go to a gynecologist or who do not get regular examinations

                                                             ii.      Most are squamous cell carcinomas since the vulva is covered by squamous cell epithelium

                                                            iii.      They tend to spread to the inguinal lymph nodes

                                                            iv.      Treatment in the past was a big incision known as “Butterfly” in which they removed the entire vulva along with the lymph nodes in the inguinal area = very bad for women as it often required skin grafts amongst other things; overall it was detrimental to the health of the woman

                                                             v.      Treatment today includes a vulvectomy (removal of the affected vulva) and/or separate excisions = women do much better

                                                           vi.      Lesions can be exophytic (going outward) or endophytic (ulcerating inward)

                                                         vii.      Non-HPV related lesions are more common in the elderly while HPV-related ones are more common in the younger population

II.                   Vaginal conditions

a.       Inflammatory à often picked up on pap smears; caused by

                                                               i.      Monilla (yeast infection)

                                                             ii.      Trichomonas – seen well with wet slide; PAP smear not that dramatic as the do not swim around

                                                            iii.       Bacterial vaginosis due to shift in the normal vaginal flora; discharge seen; can cause premature labor in pregnant women

                                                            iv.      Actinomyces – associated with intrauterine devices

                                                              v.      Herpes

b.       Malignancies – occur usually if have something else going on such as in the vulva

                                                               i.      Vaginal intraepithelial neoplasia (VAIN) – HPV-related

                                                            ii.      Invasive Squamous cell carcinoma – to diagnose as a primary cancer must rule out vulvar or cervical cancer first

                                                          iii.      Adenosis or trapping of glands that should not be there – normally there are no glands in the vagina so this is rare – associated with clear cell carcinoma caused by diethlystilbetrol (DES) use (no longer used)

                                                           iv.      Sarcoma Botryoides (Rhabdomyosarcoma of the vagina)– pediatric smooth mm sarcoma; usually seen in children under 5 yo; rare; once quite deadly – better outcomes today; clinically appear as clusters of grape

                                                              v.      Rare tumors, metastases


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