II. Definition

  1. Idiopathic (possibly autoimmune), chronic inflammatory condition of peri-mucosal skin

III. Symptoms

  1. Asymptomatic in one third of patients
  2. Pruritic genital lesions
    1. Involves vulva or foreskin
    2. May be severe enough to interfere with sleep
  3. Dysuria
  4. Painful Intercourse
    1. Dyspareunia in women
    2. Painful Erections in men
  5. Painful Defecation (if Anal Fissures present)

IV. Signs

  1. Initial
    1. Vulva or foreskin is thick and white
    2. Contiguous edema may be present
    3. Labia minora may be edematous and partially resorbed
  2. Later
    1. Vulva or foreskin in thin, Wrinkled and hypopigmented (like celophane)
    2. Bruising may be present
  3. Last
    1. Vulva or foreskin and contiguous anatomy distorted
    2. May obscure surrounding antomy
      1. Clitoris and labia minora may appear buried in women
      2. Phimosis may occur in men (may result in obstructive uropathy)

V. Labs: Biopsy

  1. Biopsy especially indicated if squamous cell hyperplasia present
  2. Risk of developing Squamous Cell Carcinoma of the vulva or foreskin is 5% in Lichen Sclerosus
  3. Also biopsy vulvar or foreskin lesions that fail to heal with management (see below)

VI. Differential Diagnosis

  1. See Pruritus Vulvae
  2. Squamous Cell Hyperplasia
  3. Squamous Cell Cancer

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

VIII. 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]

IX. Complications

  1. Squamous Cell Carcinoma (4-6% of genital Lichen Sclerosus)
    1. Genital Lichen Sclerosus is considered premalignant
    2. Penile Cancer
    3. Vulvar Cancer

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Ontology: Lichen Sclerosus et Atrophicus (C0023652)

Definition (MSH) A chronic inflammatory mucocutaneous disease usually affecting the female genitalia (VULVAR LICHEN SCLEROSUS) and BALANITIS XEROTICA OBLITERANS in males. It is also called white spot disease and Csillag's disease.
Definition (NCI) A chronic inflammatory process affecting the skin. It is characterized by the presence of white, indurated plaques, epidermal atrophy, and fibrosis of the upper dermis. It usually appears in the vulva and penis.
Concepts Disease or Syndrome (T047)
MSH D018459
ICD10 L90.0
SnomedCT 267858008, 156395005, 25674000
English Lichen Sclerosus, Lichen Sclerosus et Atrophicus, LSA, LICHEN SCLEROSUS ET ATROPHICUS, lichen sclerosus et atrophicus, lichen sclerosus et atrophicus (diagnosis), Lichen sclerosis & atrophicus, Sclerosus, Lichen, Lichen Sclerosis, Scleroses, Lichen, Sclerosis, Lichen, Lichen Sclerosis et Atrophicus, Lichen Scleroses, Lichen Sclerosus et Atrophicus [Disease/Finding], lichen et sclerosus atrophicus, sclerosus lichen, lichen et atrophicus sclerosus, atrophicus lichens sclerosis, white spot disease, lichen sclerosis atrophicus, lichen sclerosus, lichens sclerosus, Lichen sclerosis atrophicus, Lichen sclerosus et atropicus, Lichen sclerosus et atrophicus, Lichen sclerosus, White spot disease, Lichen sclerosus et atrophicus (disorder), disease (or disorder); white spot (lichen sclerosus et atrophicus), disease; white spot, lichen; sclerosus, sclerosus; lichen, white spot; disease, white spot; lichen sclerosus et atrophicus, Lichen sclerosus et atrophicus, NOS
Italian Lichen sclerosus, Lichen sclero-atrofico, Lichen scleroso, Lichen scleroso e atrofico, Lichen sclero-atrofico infantile, Lichen scleroatrofico
Dutch lichen sclerosus et atrophicus, lichen sclerosus en atrophicus, witte vlekkenziekte, aandoening; white spot (lichen sclerosus et atrophicus), disease; white spot, lichen; sclerosus, sclerosus; lichen, white spot; disease, white spot; lichen sclerosus et atrophicus, lichen sclerosus, Lichen sclerosus, Lichen sclerosus et atrophicus
French Lichen scléreux atrophique, Lichen scléreux et atrophique, Morphée en gouttes, Lichen scléreux, Lichen scléro-atrophique, Lichen scléroatrophique
Portuguese Líquen escleratrófico, Líquen escleroso e atrófico, Líquen escleroso, Líquen esclero-atrófico, Líquen Escleroso e Atrófico, Líquen Escleroso
Spanish Liquen escleroso atrófico, Liquen escleroso y atrófico, Lichen sclerosus et atrophicus, Enfermedad de las manchas blancas, liquen escleroso y atrófico (trastorno), liquen escleroso y atrófico, Liquen escleroso, Liquen Escleroso, Liquen Escleroso y Atrófico
Swedish Lichen sclerosus et atrophicus
Japanese コウカセイタイセン, コウカセイイシュクセイタイセン, 苔癬-硬化性萎縮性, 萎縮性硬化性苔癬, ハクハンビョウ, 白斑病, 硬化性苔癬, 硬化性萎縮性苔癬, 苔癬-硬化性, 苔癬-萎縮性硬化性
Czech lichen sclerosus et atrophicus, Lichen sclerosus et atrophicus, Lichen sclerosus, Nemoc bílých skvrnek
Finnish Valkojäkälä
Korean 경화위축성 태선
Polish Liszaj twardzinowy i zanikowy, Liszaj twardzinowy
Hungarian Lichen sclerosus és atrophicus, Lichen sclerosus, Lichen sclerosus et atrophicus, Lichen sclerosus et atrophichus, Fehér folt betegség
Norwegian Lichen sclerosus et atroficus
German Weisse- Flecken- Krankheit), Lichen sclerosus et atrophicus, Lichen sclerosus