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Vulvar Lichen SclerosusAka: Lichen Sclerosus, Lichen Sclerosis, Lichen Sclerosus et atrophicus

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  1. Definition
    1. Idiopathic (possibly autoimmune), chronic inflammatory condition of peri-mucosal skin
    2. Most often affects vulva, but can also affect peri-anal skin and tip of penis in men
  2. Symptoms
    1. Asymptomatic in one third of patients
    2. Vulvar Itching (may be severe enough to interfere with sleep)
    3. Dysuria
    4. Dyspareunia
    5. Painful Defecation (if Anal Fissures present)
  3. 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. 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)
  4. 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)
  5. Differential Diagnosis
    1. See Pruritus Vulvae
    2. Squamous Cell Hyperplasia
  6. Associated Conditions: Autoimmune Conditions (present in >20% of cases)
    1. Alopecia Areata
    2. Vitiligo
    3. Hypothyroidism or Hyperthyroidism
    4. Pernicious Anemia
  7. 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
      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
      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
      2. Hormonal creams (Progesterone or Testosterone) are not effective
        1. Sideri (1994) Int J Gynaecol Obstet 46:53
  8. References
    1. O'Connell (2008) Am Fam Physician 77:321
    2. Meffert (1995) J Am Acad Dermatol 32:393

Lichen Sclerosus et Atrophicus (C0023652)

Definition (MSH)A chronic, atrophic skin disease characterized by white, angular, flat, well-defined, indurated papules with an erythematous halo and follicular, black, keratotic plugs. It is the most common cause of VULVAR LICHEN SCLEROSUS in females and balanitis xerotica obliterans in males. It is called also white spot disease and Csillag's disease. (Dorland, 27th ed)
ConceptsDisease or Syndrome (T047)
ICD9701.0
MSHD018459
EnglishLichen Sclerosus, Lichen Sclerosus et Atrophicus, Lichen sclerosus et atropicus, LSA, White spot disease
Spanishliquen escleroso y atrofico
Parent ConceptsLichenoid Eruptions (C0162848), Dermatitis (C0011603), Lichen disease (C0023643), Degenerative skin disorder (C0342981)
SourcesCOSTAR, DXP, MSH, MTHICD9, NCI, NDFRT, OMIM, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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