II. Definition
- Idiopathic (possibly autoimmune), chronic inflammatory condition of peri-mucosal skin
III. Symptoms
- Asymptomatic in one third of patients
- Pruritic genital lesions
- Involves vulva or foreskin
- May be severe enough to interfere with sleep
- Dysuria
-
Painful Intercourse
- Dyspareunia in women
- Painful Erections in men
- Painful Defecation (if Anal Fissures present)
IV. Signs
- Initial
- Vulva or foreskin is thick and white
- Contiguous edema may be present
- Labia minora may be edematous and partially resorbed
- Later
- Last
V. Labs: Biopsy
- Biopsy especially indicated if squamous cell hyperplasia present
- Risk of developing Squamous Cell Carcinoma of the vulva or foreskin is 5% in Lichen Sclerosus
- Also biopsy vulvar or foreskin lesions that fail to heal with management (see below)
VI. Differential Diagnosis
- See Pruritus Vulvae
- Squamous Cell Hyperplasia
- Squamous Cell Cancer
VII. Associated Conditions: Autoimmune Conditions (present in >20% of cases)
VIII. Management
-
Topical Corticosteroids
- Initial (first 2-3 months until active inflammation has resolved)
- Level 1 High potency Corticosteroid (e.g. Temovate 0.05% ointment) applied daily
- Lorenz (1998) J Reprod Med 43:790-4 [PubMed]
- Later (maintenance)
- Taper high potency steroid to 1-2 times weekly or
- Level 5 Medium potency steroid (e.g. Valisone 0.1% cream) applied daily
- Initial (first 2-3 months until active inflammation has resolved)
- Clinic procedures for thickened lesions
- Intralesional Corticosteroid Injection (up to 10-20 mg of triamcinoline acetonide)
- Cryotherapy (one freeze per lesion)
- Other management
- Tretinoin (e.g. Retin-A) applied topically to lesions
- Hormonal creams (Progesterone or Testosterone) are not effective
IX. Complications
-
Squamous Cell Carcinoma (4-6% of genital Lichen Sclerosus)
- Genital Lichen Sclerosus is considered premalignant
- Penile Cancer
- Vulvar Cancer