Benign Vulvar Conditions

There are several benign vulvar diseases, but some of the most common are Bartholin cyst and abscess, lichen sclerosus, and lichen simplex chronicus. Bartholin cysts are formed due to an obstruction in the excretory duct that causes retention of their secretions (lubricating mucus). Bartholin cysts present as nontender fluctuant masses at the 4 and/or 8 o’clock positions in the labia. If a Bartholin cyst becomes infected, it can develop into an extremely painful abscess. Lichen sclerosus is a chronic dermatologic condition that causes progressive thinning and fibrosis of the vulvar, perineal, and perianal skin, and presents classically with itching and white plaques. Lichen simplex chronicus is a thickening of the vulvar skin due to chronic itching or rubbing, which often occurs in the setting of atopic or contact dermatitis.

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Bartholin Cysts and Abscesses

Definition

Bartholin cysts are cysts within the Bartholin gland that result from the obstruction of their excretory duct and retention of their secretory fluid. If this fluid, or the gland itself, becomes infected, a Bartholin abscess is formed.

Anatomy review of the Bartholin glands

  • Also called the greater vestibular glands
  • Paired glands located on both sides of the vestibule (space between the labia minora, which opens into the vagina)
  • Secrete lubricating mucus into the vestibule during sexual arousal
  • Normally, approximately 0.5 cm in diameter
  • Located deep to the posterior labia majora
  • Partially surrounded by vestibular bulbs and bulbospongiosus muscles
  • Ducts:
    • Approximately 2.5 cm in length
    • Open into the vestibule at 4 and 8 o’clock positions (when viewing the vaginal opening as the center of the clock face)

Epidemiology

  • Most common vulvar mass
  • Develop in approximately 2% of women of reproductive age
  • Risk factors:
    • Obesity
    • Poor hygiene
    • Shaving or waxing of pubic hair
    • Immunocompromised individuals
    • Pregnancy

Etiology and pathogenesis

  • Nonspecific inflammation or trauma of the duct → duct occlusion → mucinous fluid accumulation within the gland (Bartholin cyst)
  • Bartholin abscess:
    • Infection of a Bartholin cyst
    • Primary infection of the gland
  • Microbiology:
    • Usually polymicrobial, consisting of:
      • MRSA
      • Enteric gram-negative aerobes
      • Lower genital tract anaerobes that are found in women
    • Most common single pathogen: Escherichia coli
    • Less common, but also potentially due to STIs (e.g., Neisseria gonorrhoeae, Chlamydia trachomatis)

Clinical presentation

Signs and symptoms of Bartholin cysts:

  • Typically present as a painless, unilateral, fluctuant mass
  • Appear near the posterior vaginal introitus
  • Often 1‒3 cm in size
  • Larger cysts may cause mild discomfort (especially during intercourse or movement).

Signs and symptoms of a Bartholin abscess:

  • Unilateral palpable mass near the posterior vaginal introitus
  • May measure up to 4‒5 cm
  • Significant pain/tenderness in the vulva:
    • Acute onset
    • Often difficult to walk and sit due to pain
    • Dyspareunia (pain with intercourse)
  • Fever (possible, but uncommon)
Bleeding Bartholin gland cyst

Bartholin gland cyst:
This cyst was recently injected with local anesthetic, resulting in the bleeding seen in the image.

Image: “Procedure with CO2 laser in the treatment of a Bartholin cyst” by Speck NM, Boechat KP, Santos GM, Ribalta JC. License: CC BY 4.0, cropped by Lecturio.

Diagnosis

  • Both cysts and abscesses are diagnosed clinically.
  • Culture: any drainage or fluid
  • Indications for biopsy to rule out malignancy (rarely needed):
    • Individuals ≥ 40 years of age or postmenopausal women
    • If the mass has solid components
    • If the mass is fixed to surrounding tissue
  • Screen for STIs if the individual is at high risk.

Management

Incision and drainage:

  • Standard treatment
  • High risk of recurrence
  • To ↓ risk of recurrence, a word catheter is placed in the incision:
    • Allows continued drainage for up to 4 weeks
    • Allows the new tract to epithelialize and remain open, preventing reaccumulation of fluid
Bartholin gland cyst management

Management of a Bartholin gland abscess:
A: Incision and drainage: A scalpel is used to make an incision in the abscess wall, allowing the puss to drain
B: Word catheter placement: The catheter is placed in the abscess and provides a route for continued drainage by preventing the incision from closing
C: Word catheter within the cyst cavity: A small balloon inflated inside the cyst wall keeps the catheter in place.

Image by Lecturio.

Advanced treatment: if the cyst/abscess continues to recur or never fully resolves

  • Marsupialization: Open the cyst and suture the cyst walls to the surrounding tissue to keep it open.
  • Gland excision:
    • Definitive treatment
    • Rarely performed due to ↑ risk of complications

Antibiotics:

  • Indications:
    • Culture-positive for MRSA
    • Surrounding cellulitis
    • Immunocompromised individuals
    • Signs of systemic infection
  • NOT indicated for:
    • Cysts
    • Uncomplicated abscesses that can be treated with incision and drainage
  • Regimens should cover anaerobes and MRSA.
  • Recommended regimens include:
    • Trimethoprim-sulfamethoxazole +/- metronidazole
    • Trimethoprim-sulfamethoxazole +/- amoxicillin-clavulanate
    • Doxycycline + metronidazole

Lichen Sclerosus

Definition

Lichen sclerosus is a chronic, progressive, dermatologic condition of the vulva characterized by inflammation and epithelial thinning. As it progresses, scarring can distort the anatomy. Lichen sclerosus itself is benign, but is associated with an increased risk for vulvar squamous cell carcinoma (SCC).

Epidemiology

  • Gender: women > men
  • Age: 2 peaks (both low-estrogen states):
    • Prepubertal girls
    • Perimenopausal or post-menopausal women
  • Incidence:
    • Approximately 15 per 100,000 woman-years
    • Incidence is increasing.

Pathogenesis

  • Etiology is unknown.
  • Contributing factors likely include:
    • Genetic factors
    • Local factors (e.g., local irritation)
    • Hormonal factors
    • Immunologic abnormalities
  • Chronic, progressive process
  • Inflammation and altered fibroblast function lead to:
    • Thinning of the epidermis
    • Areas of atrophy
    • Fibrosis in the upper dermis

Clinical presentation

Symptoms:

  • Vulvar pruritus
  • Vulvar irritation or soreness
  • Dyspareunia
  • Anal discomfort:
    • Pruritus
    • Painful defecation

Signs:

  • Porcelain-white, “parchment-like” plaques (classic finding):
    • Most common on the labia
    • “Figure of 8” pattern: white plaques around the labia, perineum, and anus
  • Lesions may also appear:
    • Hemorrhagic or purpuric
    • Eroded or ulcerated
    • Hyperkeratotic
  • Areas where fissures are common:
    • Posterior fourchette
    • Perianal region
    • Interlabial folds
    • Periclitoral region
  • Excoriations, which may be associated with:
    • Mild lichenification (thickening of the epidermis)
    • Edema of the labia minora
  • Scarring → leads to “loss-of-vulvar architecture”:
    • Fusion of the labia
    • Fusion of the clitoral hood
    • Smaller introitus and perineum
  • The vagina is generally not involved.
  • Extragenital lichen sclerosus is possible, with white plaques most commonly seen on:
    • Thighs
    • Breasts
    • Shoulders, neck, and back
A 65-year-old woman with lichen sclerosus

A 65-year-old woman with lichen sclerosus showing the characteristic “figure-of-8” distribution: Typical lesions include white, flat papules.

Image: “A 65-year-old woman with lichen sclerosus” by Fistarol SK. License: CC0 1.0

Diagnosis

The diagnosis can be made clinically, although biopsies are often preferred to confirm the diagnosis.

Vulvar biopsy:

  • Gold standard for diagnosis (though not all cases require biopsy)
  • Indicated if:
    • Medical management fails.
    • Malignancy is to be excluded.
    • Clinical diagnosis is uncertain.
  • Findings include:
    • Thinned epidermal layer
    • Areas of hyperkeratosis are possible.
    • Upper dermis: homogenization of collagen with a band of lymphocytes
Histologic section of a vulvar biopsy demonstrating the characteristic findings in lichen sclerosus

Histologic section of a vulvar biopsy demonstrating the characteristic findings in lichen sclerosus: Note the thinned epidermal layer.

Image: “Lichen sclerosus, atrophic” by Ed Uthman. License: CC BY 2.0

Management

The 1st-line treatment is medical management with high-potency topical corticosteroids.

  • Clobetasol (topical):
    • Treatment of choice
    • Initial treatment: 6‒12 weeks
    • Maintenance treatment: may be lifelong
  • Good vulvar hygiene (1st line, along with clobetasol):
    • Avoid harsh soaps, shampoos, and laundry detergents.
    • Avoid excessive washing/scrubbing.
    • Gently, but fully, rinse the area with water only.
    • Apply topical emollients (e.g., vaseline, aquaphor) after rinsing (acts as a skin protectant to ↓ irritation).
    • White cotton or silk underwear (thongs, lace, or synthetic materials to be avoided)
    • Avoid tight-fitting pants.
  • Treatment resistance:
    • Triamcinolone (injected into the lesion)
    • Topical calcineurin inhibitors:
      • Tacrolimus
      • Pimecrolimus
    • Rule out Candida infection or bacterial superinfection.
    • Biopsy, if not yet done for:
      • Confirming the diagnosis
      • Ruling out malignancy
  • Other treatment options:
    • Phototherapy (limited data)
    • Topical progesterone (traditional treatment, although less effective than clobetasol)

Prognosis

  • Increased risk of SCC of the vulva → long-term follow-up is recommended
  • Remission less likely with age

Lichen Simplex Chronicus

Definition

Lichen simplex chronicus is a benign vulvar skin disorder characterized by hyperkeratosis (thickening of the skin) that occurs secondary to chronic vulvar irritation.

Epidemiology

  • The exact frequency is unknown.
  • More common in women than men (2:1)
  • Typically occurs in mid-to-late adulthood (30‒50 years of age)

Etiology

The exact pathophysiology is unknown, but anything that leads to chronic rubbing or itching of the vulva can cause lichen simplex chronicus. Common factors include:

  • Atopic dermatitis
  • Contact (irritant) dermatitis
  • Vulvar eczema
  • Insect bites
  • Psychological disorders including:
    • Anxiety/depression
    • OCD
    • Emotional stress

Clinical presentation

Signs and symptoms include:

  • Intense pruritus:
    • Often worse at night or when still/quiet
    • Usually intermittent
    • May be described as a burning sensation
  • Skin lesions:
    • Well-demarcated, dry, patchy plaques
    • Skin is thick, scaly, firm, and/or rough.
    • Slightly erythematous
  • Accentuation of normal skin markings
  • Change in skin pigmentation (typically hyperpigmentation)
  • Excoriations
  • Extragenital lesions are possible:
    • Head, scalp, and neck
    • Hands and arms
    • Legs and ankles
Lichen simplex chronicus of the hand

Lichen simplex chronicus of the hand

Image: “A plaque of lichen simplex chronicus” by kilbad. License: CC BY 3.0

Diagnosis

The diagnosis is primarily clinical; however, a biopsy is frequently warranted to confirm the diagnosis and exclude malignancy.

  • Biopsy findings include:
    • Hypertrophic epidermal layer
    • Prominent granular cell layer
    • Acanthosis
    • Spongiosis
    • Papillary dermal fibrosis
    • Perivascular and interstitial inflammation
  • Vulvovaginitis should be excluded by:
    • Microscopy of vaginal fluid:
      • KOH prep
      • Wet mount (normal saline)
    • Fungal cultures
    • Point-of-care tests for bacterial vaginosis (BV)

Management

  • Good vulvar hygiene
  • Medical management:
    • Topical corticosteroids:
      • Clobetasol: for a shorter initial course
      • Lower potency steroids may be used for a longer term as maintenance therapy.
    • Topical emollients
    • Antibiotics, if cellulitis is present
    • Antihistamines for symptomatic treatment of pruritus:
      • Diphenhydramine
      • Hydroxyzine
  • Other treatment options:
    • Phototherapy (limited data)
    • Psychotherapy +/- antidepressants, as indicated
    • Surgical excision may be considered for small, localized lesions that do not respond to treatment.

Prognosis

  • Good with treatment
  • Lesions may resolve completely.
  • Possible complications:
    • Mild scarring and pigment changes
    • Secondary infections
    • Malignant transformation (rare)

References

  1. Quinn, A. (2017). Bartholin gland diseases. Medscape. Retrieved Sep 1, 2021, from https://emedicine.medscape.com/article/777112-overview 
  2. Chen, K.T. (2021). Bartholin gland masses: diagnosis and management. UpToDate. Retrieved Sep 1, 2021, from https://www.uptodate.com/contents/bartholin-gland-masses-diagnosis-and-management 
  3. Lazenby, G., Thurman, A., Soper, D.E. (2021). Vulvar abscess. UpToDate. Retrieved Sep 1, 2021, from https://www.uptodate.com/contents/vulvar-abscess 
  4. Cooper, S., Arnold, S. (2021). Vulvar lichen sclerosis. UpToDate. Retrieved Sep 1, 2021, from https://www.uptodate.com/contents/vulvar-lichen-sclerosus 
  5. Pappas-Taffer, L. (2020). Lichen sclerosus. Medscape. Retrieved Sep 1, 2021, from https://emedicine.medscape.com/article/1123316-overview#a4 
  6. Schoenfeld, J. (2020). Lichen simplex chronicus. Medscape. Retrieved Sep 1, 2021, from https://emedicine.medscape.com/article/1123423-overview 
  7. Charifa, A. (2021). Lichen simplex chronicus. StatPearls. Retrieved Sep 1, 2021, from https://www.statpearls.com/articlelibrary/viewarticle/24257/ 
  8. Larrabee, R., Kylander, D.J. (2001). Benign vulvar disorders. Identifying features, practical management of nonneoplastic conditions and tumors. Postgrad Med. 109, 151-4, 157-9, 163-4. https://pubmed.ncbi.nlm.nih.gov/11381665/
  9. Sinha, P., Sorinola, O., Luesley, D.M. (1999). Lichen sclerosus of the vulva. Long-term steroid maintenance therapy. J Reprod Med. 44, 621-4. https://pubmed.ncbi.nlm.nih.gov/10442326/
  10. Smith, Y.R., Haefner, H.K. Vulvar lichen sclerosus: Pathophysiology and treatment. Am J Clin Dermatol. 2004. 5, 105-25. https://link.springer.com/article/10.2165%2F00128071-200405020-00005

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