Vulvar Lichen Sclerosus

Aka: Vulvar Lichen Sclerosus

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II. Definitions

  1. Vulvar Lichen Sclerosus
    1. Idiopathic (possibly autoimmune), chronic inflammatory condition of peri-mucosal skin
    2. Most often affects vulva, but can also affect peri-anal skin and foreskin in men

III. Epidemiology

  1. Prevalence: 1.7%
  2. Incidence: 14 to 22 cases per 100,000 person-years

IV. Pathophysiology

  1. Chronic inflammatory dermatitis of the vulvar and anogenital regions
  2. Associated with both Autoimmune Conditions as well as low Estrogen states (e.g. Menopause)

V. Symptoms

  1. Asymptomatic in one third of patients
  2. Vulvar Itching (may be severe enough to interfere with sleep)
  3. Dyspareunia
  4. Painful Defecation (if Anal Fissures present)
  5. Dysuria

VI. Signs

  1. Initial
    1. Vulva are thick and white
    2. Labia minora may be edematous and partially resorbed
  2. Later
    1. Vulva are thin, Wrinkled and hypopigmented (like "Cigarette paper")
    2. White and thin Plaques form on vulva, perineum and perinanal area (figure-of-eight)
    3. Bruising may be present
  3. Last
    1. Vulva and contiguous anatomy distorted
    2. Clitoris and Labia minora may not be visible (buried in surrounding tissue)

VII. Labs: Biopsy

  1. Biopsy especially indicated if squamous cell hyperplasia present
  2. Risk of developing Squamous Cell Carcinoma of the vulva is 5% in Lichen Sclerosus
  3. Also biopsy vulvar lesions that fail to heal with management (see below)

VIII. Differential Diagnosis

  1. See Pruritus Vulvae
  2. Squamous Cell Hyperplasia

IX. Associated Conditions: Autoimmune Conditions (present in >20% of cases)

  1. Alopecia Areata
  2. Vitiligo
  3. Hypothyroidism or Hyperthyroidism
  4. Pernicious Anemia

X. Complications

  1. Squamous Cell Carcinoma of the vulva

XI. Management

  1. Topical Corticosteroids
    1. General
      1. Ointments are preferred over creams
    2. Adults - Initial (first 3-4 months)
      1. Level 1 High potency Corticosteroid (e.g. Temovate 0.05% ointment)
        1. Start at twice daily for the first 1-2 months until active inflammation has resolved
        2. Taper to 1-2 times weekly for another 2 months, then switch to lower potency steroid
          1. Alternatively, switch to lower potence steroid daily as below
      2. References
        1. Cooper (2004) Arch Dermatol 140:702-6 [PubMed]
        2. Lorenz (1998) J Reprod Med 43:790-4 [PubMed]
    3. Adults - Later (maintenance)
      1. Taper high potency steroid to 1-2 times weekly (see above) or
      2. Level 5 Medium potency steroid (e.g. Valisone 0.1% cream) applied daily
    4. Children
      1. Hydrocortisone (2.5%) topically
  2. Additional measures for refractory lesions
    1. Topical Calcineurin Inhibitor (e.g. Pimecrolimus 1% Cream)
  3. Clinic procedures for thickened lesions
    1. Intralesional Corticosteroid Injection (up to 10-20 mg of triamcinoline acetonide)
      1. Avoid total vulvar injection >40 mg triamcinoline acetonide
      2. Mazdisnian (1999) J Reprod Med 44:332-4 [PubMed]
    2. Fractional CO2 Laser Therapy
    3. Cryotherapy (one freeze per lesion)
  4. Other management
    1. Tretinoin (e.g. Retin-A) applied topically to lesions
      1. Bracco (1993) J Reprod Med 38:37-40 [PubMed]
    2. Oral Cyclosporine
    3. Oral Methotrexate
    4. Hormonal creams (Progesterone or Testosterone) are not effective
      1. Sideri (1994) Int J Gynaecol Obstet 46:53-6 [PubMed]
  5. Monitoring
    1. Reevaluate at least every 6 months

XII. References

  1. O'Connell (2008) Am Fam Physician 77:321-30 [PubMed]
  2. Meffert (1995) J Am Acad Dermatol 32:393-416 [PubMed]
  3. Ringel (2020) Am Fam Physician 102(9):550-7 [PubMed]

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