II. Definition

  1. Idiopathic (possibly autoimmune), chronic inflammatory condition of peri-mucosal skin

III. Symptoms

  1. Asymptomatic in one third of patients
  2. Pruritic genital lesions
    1. Involves vulva or foreskin
    2. May be severe enough to interfere with sleep
  3. Dysuria
  4. Painful Intercourse
    1. Dyspareunia in women
    2. Painful Erections in men
  5. Painful Defecation (if Anal Fissures present)

IV. Signs

  1. Initial
    1. Vulva or foreskin is thick and white
    2. Contiguous edema may be present
    3. Labia minora may be edematous and partially resorbed
  2. Later
    1. Vulva or foreskin in thin, Wrinkled and hypopigmented (like celophane)
    2. Bruising may be present
  3. Last
    1. Vulva or foreskin and contiguous anatomy distorted
    2. May obscure surrounding antomy
      1. Clitoris and labia minora may appear buried in women
      2. Phimosis may occur in men (may result in obstructive uropathy)

V. Labs: Biopsy

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

VI. Differential Diagnosis

  1. See Pruritus Vulvae
  2. Squamous Cell Hyperplasia
  3. Squamous Cell Cancer

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

VIII. Management

  1. Topical Corticosteroids
    1. Initial (first 2-3 months until active inflammation has resolved)
      1. Level 1 High potency Corticosteroid (e.g. Temovate 0.05% ointment) applied daily
      2. Lorenz (1998) J Reprod Med 43:790-4 [PubMed]
    2. Later (maintenance)
      1. Taper high potency steroid to 1-2 times weekly or
      2. Level 5 Medium potency steroid (e.g. Valisone 0.1% cream) applied daily
  2. Clinic procedures for thickened lesions
    1. Intralesional Corticosteroid Injection (up to 10-20 mg of triamcinoline acetonide)
      1. Mazdisnian (1999) J Reprod Med 44:332-4 [PubMed]
    2. Cryotherapy (one freeze per lesion)
  3. Other management
    1. Tretinoin (e.g. Retin-A) applied topically to lesions
      1. Bracco (1993) J Reprod Med 38:37-40 [PubMed]
    2. Hormonal creams (Progesterone or Testosterone) are not effective
      1. Sideri (1994) Int J Gynaecol Obstet 46:53-6 [PubMed]

IX. Complications

  1. Squamous Cell Carcinoma (4-6% of genital Lichen Sclerosus)
    1. Genital Lichen Sclerosus is considered premalignant
    2. Penile Cancer
    3. Vulvar Cancer

Images: Related links to external sites (from Bing)

Related Studies