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)

X. Complications

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

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Ontology: Vulvar Lichen Sclerosus (C0022783)

Definition (NCI) A chronic inflammatory disorder of unknown etiology that affects the vulva. It is characterized by the development of white elevated plaques in the vulva. Histologically there is marked subepithelial fibrosis. Clinical manifestations include pruritus, dysuria, and dyspareunia.
Definition (MSH) Atrophy and shriveling of the SKIN of the VULVA that is characterized by the whitish LICHEN SCLEROSUS appearance, inflammation, and PRURITUS.
Concepts Disease or Syndrome (T047)
MSH D007724
ICD10 N90.4
SnomedCT 73057003, 156019006, 198379009, 266666006, 26348009, 198380007
English Vulvae, Kraurosis, Leukoplakic vulvitis, VULVAL LEUKOPLAKIA, kraurosis of vulva (diagnosis), kraurosis of vulva, leukoplakia of vulva, leukoplakia of vulva (diagnosis), Vulval leukoplakia, Vulvar Lichen Sclerosus [Disease/Finding], leukoplakias vulval, vulva leukoplakia, leukoplakia vulva, leucoplakia vulva, kraurosis vulvae, lichen sclerosus of vulva, vulval leukoplakia, Kraurosis vulvae (disorder), Kraurosis - vulva, Vulva - kraurosis, Kraurosis of vulva (disorder), Kraurosis vulvae, Kraurosis of vulva, Leukoplakia of vulva, Lichen sclerosus et atrophicus of the vulva, Lichen sclerosus of vulva, Leucoplakia of vulva, Leukoplakia of vulva (disorder), Lichen sclerosus et atrophicus of the vulva (disorder), Breisky, kraurosis; vulvae, leukokraurosis vulvae, leukoplakia; vulva, leukoplakic; vulvitis, vulva; kraurosis, vulva; leukoplakia, vulvae; kraurosis, vulvitis; leukoplakic, Breisky's disease, Leukokraurosis, Kraurosis vulvae -RETIRED-, Leucoplakia of Vulva, Vulval Leukoplakia, Breisky's Disease, Kraurosis Vulvae, Vulvar Lichen Sclerosus, Lichen Sclerosus of Vulva, Lichen Sclerosus, Vulvar, Sclerosus, Vulvar Lichen, Vulva Lichen Sclerosus
Italian Leucoplachia della vulva, Vulvite leucoplasica, Kraurosis vulvae, Craurosi vulvare
Dutch vulvitis met leukoplakie, kraurosis vulvae, kraurosis; vulvae, leukoplakie; vulva, leukoplakie; vulvitis, vulva; kraurosis, vulva; leukoplakie, vulvae; kraurosis, vulvitis; leukoplakie, Leukoplakie van vulva, vulva leukoplakie, Kraurosis vulvae, Leukoplakie, vulva-
French Vulvite leucoplasique, LEUCOPLASIE VULVAIRE, Leucoplasie vulvaire, Kraurosis vulvaire, Kraurosis vulvae, Lichen scléreux de la vulve
Portuguese Vulvite leucoplásica, LEUCOPLASIA VULVAR, Craurose vulvar, Leucoplasia vulvar, Líquen Escleroso Vulvar
Spanish Vulvitis leucoplásica, Craurosis vulvar, leucoplasia de la vulva, craurosis vulvar, vulvitis leucoplásica, craurosis de la vulva - RETIRADO - (concepto no activo), enfermedad de Breisky, leucocraurosis, craurosis de la vulva - RETIRADO -, craurosis vulvar (trastorno), leucoplasia de vulva (trastorno), leucoplasia de vulva, liquen escleroso y atrófico de la vulva (trastorno), liquen escleroso y atrófico de la vulva, liquen escleroso y atrófico vulvar, Leucoplasia vulvar, Liquen Escleroso Vulvar
German Lichen sclerosus der Vulva, Kraurosis vulvae, Leukoplakie der Vulva, Craurosis vulvae
Japanese 白斑症性外陰部炎, 外陰白斑病, ハクハンショウセイガイインブエン, ガイインハクハンビョウ, ガイインイシュクショウ, 陰門萎縮症, 外陰萎縮症, 外陰ジストロフィー
Swedish Lichen sclerosus i vulva
Czech krauróza vulvy, vulvární lichen sclerosus, craurosis vulvae, vulva - lichen sclerosus, Leukoplakie vulvy, Leukoplakická vulvitida, Krauróza vulvy
Finnish Vulvan krauroosi
Korean 외음부의 백반증
Polish Marskość sromu
Hungarian Vulva leukoplakia, Kraurosis vulvae, Leukoplakiás vulvitis
Norwegian Lichen sclerosus i vulva, Kraurosis vulvae, Kraurose i vulva, Kraurose i skjedeinngangen