II. Definition

  1. Idiopathic (possibly autoimmune), chronic inflammatory condition of peri-mucosal skin
  2. Typically vulvar involvement in women is more common

III. Epidemiology

  1. Average age of onset 42 years
  2. Prevalence: One in 300 males

IV. Symptoms

  1. Asymptomatic in one third of patients
  2. Pruritic foreskin
    1. May bleed
    2. May be severe enough to interfere with sleep
  3. Dysuria
  4. Urinary obstruction (long-standing cases)
  5. Painful Erections
  6. Painful Defecation (if Anal Fissures present)

V. Signs

  1. Initial
    1. Glans and foreskin is thick and white
    2. Contiguous edema may be present
  2. Later
    1. Foreskin in thin, Wrinkled and hypopigmented (like cellophane)
    2. Bruising may be present
  3. Last
    1. Foreskin and contiguous anatomy distorted
    2. May obscure surrounding antomy
      1. Phimosis may occur in men (may result in obstructive uropathy)

VI. Labs: Biopsy

  1. Biopsy especially indicated if squamous cell hyperplasia present
  2. Risk of developing Squamous Cell Carcinoma of the foreskin is 4-6% in Lichen Sclerosus
  3. Biopsy foreskin lesions that fail to heal with management (see below)

VII. Differential Diagnosis

  1. Penile Squamous Cell Cancer in-situ
  2. Penile Squamous Cell Cancer
  3. Scleroderma

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

IX. Management (much of the supporting research was done in women with Vulvar Lichen Sclerosus)

  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 topical management
    1. Tacrolimus or Pimecrolimus
    2. Tretinoin (e.g. Retin-A) applied topically to lesions
      1. Bracco (1993) J Reprod Med 38:37-40 [PubMed]
    3. Hormonal creams (Progesterone or Testosterone) are not effective
      1. Sideri (1994) Int J Gynaecol Obstet 46:53-6 [PubMed]
  4. Other systemic medications (severe refractory cases)
    1. Systemic Retinoids
    2. Methotrexate
  5. Surgical management
    1. Circumcision (if not already circumsized)
    2. Reconstructive surgery (severe cases)

X. Complications

  1. Penile squamous cell cancer
    1. Genital Lichen Sclerosus is considered premalignant (4-6% squamous cell cancer Incidence)
  2. Phimosis
  3. Painful Erection
  4. Urinary obstruction

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