Gianani Diseases of the vulva

Question 1

Diseases of the vulva

Answer 1

Diseases of the vulva in the aggregate constitute only a small fraction of gynecologic practice. Many inflammatory diseases that affect skin elsewhere on the body also occur on the vulva, such as psoriasis, eczema, and allergic dermatitis.
Infection
Non neoplastic epithelial disorders
Neoplastic and pre-neoplastic disorders.

Question 2

Non-neoplastic epithelial disorders

Answer 2

Lichen Sclerosus et Atrophicus

Squamous Cell Hyperplasia

Question 3

Lichen Sclerosus et Atrophicus

Answer 3

Lichen sclerosis presents as smooth, white plaques or macules. Histo­logically the lesion is characterized by marked thinning of the epidermis, excessive keratinization (hyperkeratosis); sclerotic changes of the superficial dermis; and a bandlike lymphocytic infiltrate in the underlying dermis. The disease occurs in all age groups but is most common in postmenopausal women

Question 4

Squamous Cell Hyperplasia

Answer 4

nonspecific condition resulting from rubbing or scratching of the skin to relieve pruritus. Clinically it presents as leukoplakia and histologic examination reveals thickening of the epidermis (acanthosis), and hyperkeratosis

Question 5

Squamous cell Carcinoma

Answer 5

uncommon and usually occurs in older women.
There ARE TWO MAIN TYPES of vulvar SCC.
1st type: Basaloid and warty carcinomas are related to HPV (HPV16), are less common and occur in younger women.
2nd type: Keratinizing squamous cell carcinoma (not HPV related) occurs in older women and is more common.

Question 6

Vulvar Intraepithelial Neoplasia (VIN).

Answer 6

There are two types of vulvar intraepithelial neoplasia (pre-neoplastic and in situ lesion):

1st Type: Classic VIN.
2nd Type: Differentiated VIN.
Classic VIN presents either as a discrete white (hyperkeratotic) or a slightly raised, pigmented lesion. Microscopically, it is characterized by epidermal thickening, nuclear atypia, increased mitoses, and lack of cellular maturation.

Differentiated VIN is characterized by marked atypia of the basal layer of the squamous epithelium and normal-appearing differentiation of the more superficial layers. Invasive keratinzing squamous cell carcinomas that arise in differentiated VIN contain nests and tongues of malignant squamous epithelium with prominent central keratin pearls

Question 7

Vulvar Intraepithelial Neoplasia (Classic Type)- histology

Answer 7

Vulvar intraepithelial neoplasia, classic type. Nuclear enlargement, nuclear hyperchromasia, apoptosis, and mitotic activity are present in all levels of the epithelium.

Question 8

Glandular neoplastic lesions of the vulva

Answer 8

Papillary Hydroadenoma.

Extramammary Paget Disease

Question 9

Developmental Anomalies of the Vagina

Answer 9

Septate, or double, vagina

Adenosis

Question 10

Septate, or double, vagina

Answer 10

uncommon anomaly that arises from a failure of müllerian duct fusion and is accompanied by a double uterus (uterus didelphys). These and other anomalies of the external genitalia may be the manifestations of genetic syndromes, in utero exposure to diethylstilbestrol (DES, used to prevent threatened abortions in the 1940s through 1960s), or other unknown factors that perturb reciprocal epithelial-stromal signaling during fetal development.

Question 11

Adenosis

Answer 11

• embryonal development: vagina is initially covered by columnar, endocervical-type epithelium.–> replaced by squamous epithelium advancing upwards from the urogenital sinus.
• Small patches of residual glandular epithelium may persist into adult life = vaginal adenosis.
• red, granular areas that stand out from the surrounding normal pale-pink vaginal mucosa.
• columnar mucinous epithelium indistinguishable from endocervical epithelium.
• found in only a small percentage of adult women, but has been reported in 35% to 90% of women exposed to DES in utero.
• Rare cases of clear cell carcinoma arising in DES-related adenosis were recorded in teenagers and young adult women in the 1970s and 1980s, resulting in discontinuation of DES treatment

Question 12

Pre-malignant and malignant neoplasms of the vagina.

Answer 12

Virtually all primary carcinomas of the vagina are squamous cell carcinomas associated with high risk HPVs. It is an extremely uncommon cancer (about 0.6 per 100,000 women yearly) that accounts for about 1% of malignant neoplasms in the female genital tract. The greatest risk factor is a previous carcinoma of the cervix or vulva; 1% to 2% of women with an invasive cervical carcinoma eventually develop a vaginal squamous cell carcinoma. Squamous cell carcinoma of the vagina arises from a premalignant lesion, vaginal intraepithelial neoplasia, analogous to cervical squamous intraepithelial lesions. Most often the invasive tumor affects the upper vagina, particularly the posterior wall at the junction with the ectocervix. The lesions in the lower two thirds of the vagina metastasize to the inguinal nodes, whereas lesions in the upper vagina tend to spread to regional iliac nodes.

Question 13

. Embryonal Rhabdomyosarcoma

Answer 13

Also called sarcoma botryoides, this uncommon vaginal tumor composed of malignant embryonal rhabdomyoblasts is most frequently found in infants and in children younger than 5 years of age. These tumors tend to grow as polypoid, rounded, bulky masses that have the appearance and consistency of grapelike clusters (hence the designation botryoides, or grapelike.

Question 14

Causes of Vaginal and Vulvar Pruritus

Answer 14

Vulvovaginitis
Atrophic Vaginitis
Vulvovaginal Candidiasis

Question 15

Vulvovaginitis

Answer 15

• erythema, itching, burning, irritation, soreness, swelling, and/or discharge.
• most common in prepubertal girls and is one of the most common gynecological complaints in this age range.

Nonspecific vulvovaginitis:
This is the most common form and may be due to inadequate hygiene or intravaginal foreign body.
Risk factors: inadequate hand washing and difficulties in cleansing after toileting, tight-fitting underwear, perfumes or other irritants.

Infectious vulvovaginitis:
The most common causes are Streptococcus pyogenes (group A beta hemolytic), Haemophilus influenzae, and Enterobious Vermicularis.

Question 16

Atrophic Vaginitis

Answer 16

Atrophic vaginitis refers to anatomic changes in the vaginal tract commonly due to natural or induced menopause or other conditions associated with hypoestrogenic states.
Symptoms due to atrophic vaginal changes are reported in 20%-45% of postmenopausal women and include vaginal dryness, irritation, pruritus, burning, dysuria, dyspareunia, vaginal discharge, or bleeding.

Question 17

Vulvovaginal Candidiasis.

Answer 17

• Candida albicans or other Candida species.
• thick white vaginal discharge, often accompanied by vulvar pruritus and irritation, external dysuria, or dyspareunia.
• white “cottage cheese-like” vaginal discharge,

In a symptomatic woman:
wet preparation (saline or 10% potassium hydroxide) or Gram stain of vaginal discharge showing yeasts, hyphae, or pseudo hyphae
culture or other test yielding positive results for a yeast species

• needs to be distinguished from other common causes of vaginitis such as bacterial vaginosis or trichomoniasis.

Question 18

Dyspareunia

Answer 18

Sexual pain disorders include dyspareunia and vaginismus (usually not caused by a an histopathological lesion).
Dyspareunia may be associated with various medical conditions, medications, and/or underlying psychological etiologies

medical conditions associated with superficial (entry) dyspareunia include:
provoked vestibulodynia
vulvodynia
vaginismus 
vulvitis or vulvovaginitis
condylomas
postoperative scarring of vagina, including shortening of vaginal length during abdominal hysterectomy
Bartholin gland cyst or abscess
chronic vulvar dermatoses, including
lichen planus
lichen simples chronicus
lichen sclerosus 
Psoriasis 
condyloma acuminatum due to human papillomavirus (HPV)
herpes simplex virus  outbreak

Question 19

Herpes Virus Infection

Answer 19

HSV-1: oropharyngeal infection.
HSV-2: genital mucosa and skin.
Both viruses can however cause oral and genital infection.
1/3 of newly infected individuals are symptomatic.
Red papules> vesicles > painful coalescent ulcers (see below). Spontaneous healing 1 to 3 weeks followed by latent infection in the regional lumbosacral nerve ganglia.
Sexual transmission mainly during the active phase but possible also in the latent phase (subclinical virus shedding).

Question 20

The gravest consequence of HSV infection is

Answer 20

transmission in the neonate during birth. Active infection during delivery (highest risk of neonatal infection) warrants cesarean section.

Question 21

Vulvodynia

Answer 21

vulvar pain, often characterized as burning, stinging, irritating, or raw, lasting for > 3 months without an obvious etiology.
Women of all ages, reproductive stages, and ethnicities may be affected, but vulvodynia may be most common in women aged 20-40 years.
Women with provoked vulvodynia may report a sensation that something is blocking the vagina or that the vagina is too small when penetration is attempted.
The constant burning pain often reported by women with generalized unprovoked vulvodynia may be present regardless of sexual activity.
Comorbid mood or pain disorders may increase the risk of vulvodynia.
Sexually transmitted infections are not associated with an increased risk of vulvodynia.

Question 22

endometriosis summary

Answer 22

Endometriosis is defined as endometrial glands and stroma outside of the uterus. The “ectopic” endometrial tissue may undergo cyclic bleeding.
▪ Most common sites of endometriosis are within the abdominal cavity, but occasionally it is found at distant sites.
▪ Several theories (regurgitation, metaplasia, metastasis, and stem cell origin) are proposed to explain the distribution of endometriosis.
▪ It commonly results in dysmenorrhea, pelvic pain, and infertility.

Question 23

Endometrial Hyperplasia summary

Answer 23

Endometrial hyperplasia is an important cause of abnormal bleeding and a frequent precursor to the most common type of endometrial carcinoma. It is defined as an increased proliferation of the endometrial glands relative to the stroma, resulting in an increased gland-to-stroma ratio when compared with normal proliferative endometrium.
Endometrial hyperplasia is associated with prolonged estrogenic stimulation of the endometrium, which can be due to anovulation, increased estrogen production from endogenous sources, or exogenous estrogen. Associated conditions include: Obesity (peripheral conversion of androgens to estrogens), Polycystic ovarian syndrome, Granulosa cell tumors of the ovary, Excessive ovarian cortical function, estrogen replacement therapy.
Endometrial hyperplasia can be divided in Non-atypical Hyperplasia and Atypical Hyperplasia.

Question 24

Bicornuate uterus

Answer 24

(uterus with two horns).
Only the upper part of that part of the Müllerian system that forms the uterus fails to fuse, thus the caudal part of the uterus is normal, the cranial part is bifurcated. The uterus is “heart-shaped”. Potential cause of second trimester spontaneous abortion