Gynecology Book

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Vulvar Lichen Sclerosus

Aka: Vulvar Lichen Sclerosus
  1. See Also
    1. Lichen Sclerosus
    2. Penile Lichen Sclerosus
  2. 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 foreskin in men
  3. 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)
  4. 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)
  5. 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)
  6. Differential Diagnosis
    1. See Pruritus Vulvae
    2. Squamous Cell Hyperplasia
  7. Associated Conditions: Autoimmune Conditions (present in >20% of cases)
    1. Alopecia Areata
    2. Vitiligo
    3. Hypothyroidism or Hyperthyroidism
    4. Pernicious Anemia
  8. 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]
  9. References
    1. O'Connell (2008) Am Fam Physician 77:321-30 [PubMed]
    2. Meffert (1995) J Am Acad Dermatol 32:393-416 [PubMed]

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
Russian VUL'VY KRAUROZ, ATROFIIA VUL'VY PROGRESSIRUIUSHCHAIA DIFFUZNAIA, АТРОФИЯ ВУЛЬВЫ ПРОГРЕССИРУЮЩАЯ ДИФФУЗНАЯ, ВУЛЬВЫ КРАУРОЗ
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
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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