Pathology

Pathology/C/70

Diseases of the vulva and vagina

外陰・膣の疾患

1. Vulva — Vulvitis

Definition

  • Inflammation of external female genitalia (labia, clitoris, vestibule).

A) Contact dermatitis

  • Reactive inflammation to exogenous source.
  • Contact irritant: soaps, detergents, antiseptics.
  • Contact allergic: creams, perfumes.
  • Most common cause of vulvar pruritus.

B) Bacterial infections

  • Gonorrhea (N. gonorrhoeae) — affects entire gynecologic tract except vagina (only children get vaginitis); causes infertility.
  • Syphilis (T. pallidum) — primary chancre, secondary condyloma lata.

C) Viral infections

  • Herpes (HSV-2 in young women) — STD; labial papules → vesicles → ulcers.
  • Condyloma acuminatum (HPV 6/11).

D) Fungal

  • Candidiasis (Candida albicans) — vulvovaginitis; white patches + leukorrhea (thick whitish/yellow discharge) + itching.

2. Non-Neoplastic Epithelial Disorders

  • Vulvar mucosa: atrophic thinning or hyperplastic thickening.
  • Macroscopically: white de-pigmented lesions → leukoplakia.

A) Lichen sclerosus

  • Consequence of chronic irritation (scratching).
  • Thinning of epidermis + dermal fibrosis + dermal inflammation.
  • Smooth white plaques/papules that merge over time.
  • Postmenopausal women; autoimmune origin.
  • ↑ Risk of squamous cell carcinoma in non-HPV setting.

B) Lichen simplex chronicus

  • End-stage of inflammatory dermatoses.
  • Epidermal thickening + hyperkeratosis + expansion of stratum granulosum.
  • Clinically: leukoplakia.
  • Most common premenarchal + postmenopausal.
  • Atrophy + stiffening → constricted vaginal orifice.
  • Squamous cell carcinoma can arise.

3. Vulvar Tumors

A) Bartholin’s cyst

  • Bartholin’s glands secrete mucus to lubricate vagina.
  • Duct obstruction (often after gonorrhea or acute inflammation) → fluid-filled cyst → abscess.
  • Painful; often women ≥ 40 yr.
  • Excision in older women due to risk of adenoid cystic carcinoma.

B) Condylomas

  • Anogenital warts; in moist vulva environment → large.
  • Cause: low-risk HPV (HPV 6/11).
  • Two forms:
    • Condyloma latasecondary syphilis; flat minimally elevated.
    • Condyloma acuminatumSTD; papillary; histology: koilocytes + hyperkeratosis + fibrovascular cores.
  • Not pre-cancerous (low-risk HPV) but may coexist with VIN1 / cervical lesions.

C) Squamous cell carcinoma of vulva (90 % of vulvar carcinomas)

  • Mean age 60–74.
  • Two pathways:
Feature HPV-related SCC Non-HPV-related SCC
Age Younger; smokers Older women
Precursor VIN II/III, high-risk HPV (HPV-16) Lichen sclerosus (years)
VIN changes + Often absent
Progression Slow; few progress Frequent progression
Prognosis Better Poor

D) Extramammary Paget disease

  • Intraepithelial form of carcinoma (analogous to breast Paget).
  • Primary: cutaneous origin, may have invasive component.
  • Secondary: from underlying anal/rectal/bladder carcinoma.
  • Intraepidermal proliferation of mucinous (glandular) cells.
  • Clinical: red, scaly, crusted plaque.
  • Indistinct borders → frequent recurrences.

4. Vagina

  • Rarely site of primary disease; usually secondarily involved by adjacent cancers/infections.

Vaginitis (infectious causes)

  • Produces leukorrhea (whitish/yellowish discharge).
  • Often part of normal flora becoming pathogenic in immunocompromise, antibiotics, HIV.
Organism Features
Candida albicans Yeast; vulvovaginitis; white “cottage cheese” discharge • itching
Trichomonas vaginalis Large flagellated protozoan; gray-green frothy purulent discharge; “strawberry cervix”; mucosal-only inflammation
Gardnerella vaginalis Bacterial vaginosis; gray fishy-odor discharge; clue cells

Vaginal Tumors

A) Vaginal intraepithelial neoplasia (VAIN) + SCC

  • Extremely uncommon; usually > 60 yr.
  • VAIN is HPV-associated precursor.
  • Invasive SCC of vagina = HPV-associated in > 50 %.

B) Vaginal clear cell adenocarcinoma

  • Young women, late teens to early 20s.
  • Classic association: maternal diethylstilbestrol (DES) exposure during pregnancy.
  • Sometimes presents in 3rd–4th decade.
  • ~1/3 arise in cervix.
  • Arises from precursor vaginal adenosis — small glandular/microcystic inclusions, red granular foci on vaginal mucosa.

💡 High-yield: Vulvitis: contact dermatitis (#1 pruritus), gonorrhea, HSV-2 ulcers, candidiasis (vulvovaginitis + leukorrhea). Non-neoplastic epithelial disorders: lichen sclerosus (thin epidermis + autoimmune + postmenopausal → ↑ SCC risk) vs lichen simplex chronicus (thick epidermis + hyperkeratosis). Bartholin cyst = duct obstruction post-gonorrhea. Condyloma acuminatum = HPV 6/11 + koilocytes. Vulvar SCC: HPV-related (younger, HPV-16, VIN, better prognosis) vs non-HPV (older, lichen sclerosus, poor). Vaginal clear cell adenocarcinoma = DES exposure in utero; precursor = vaginal adenosis. Trichomonas = strawberry cervix + gray-green discharge.