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. Squamouse 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] (or open in [QxMD Read])
    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] (or open in [QxMD Read])
    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] (or open in [QxMD Read])
    2. Hormonal creams (Progesterone or Testosterone) are not effective
      1. Sideri (1994) Int J Gynaecol Obstet 46:53-6 [PubMed] (or open in [QxMD Read])

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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 25674000, 156395005, 267858008
English Lichen Sclerosus, Lichen Sclerosus et Atrophicus, Lichen sclerosus et atrophicus, NOS, 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, lichens sclerosus, Lichen sclerosis atrophicus, 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 atropicus, lichen sclerosus
Italian Lichen sclerosus, Lichen sclero-atrofico infantile, Lichen sclero-atrofico, Lichen scleroso, Lichen scleroso e atrofico, Lichen scleroatrofico
Dutch witte vlekkenziekte, lichen sclerosus et atrophicus, lichen sclerosus en atrophicus, 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 Morphée en gouttes, Lichen scléreux atrophique, Lichen scléreux et atrophique, Lichen scléreux, Lichen scléro-atrophique, Lichen scléroatrophique
German Weisse- Flecken- Krankheit), Lichen sclerosus et atrophicus, Lichen sclerosus
Portuguese Líquen escleratrófico, Líquen escleroso, Líquen escleroso e atrófico, Líquen esclero-atrófico, Líquen Escleroso e Atrófico, Líquen Escleroso
Spanish Liquen escleroso atrófico, Liquen escleroso y atrófico, Enfermedad de las manchas blancas, Lichen sclerosus et atrophicus, Lichen sclerosus et atropicus, liquen escleroso y atrófico (trastorno), liquen escleroso y atrófico, Liquen escleroso, Liquen Escleroso, Liquen Escleroso y Atrófico, Liquen Escleroso y Atrofico
Japanese 白斑病, ハクハンビョウ, 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, 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
Swedish Lichen sclerosus et atrophicus
Czech lichen sclerosus et atrophicus, Lichen sclerosus et atrophicus, Nemoc bílých skvrnek, Lichen sclerosus
Finnish Valkojäkälä
Russian LISHAI SKLEROTICHESKII, LISHAI SKLEROTIKOATROFICHESKII, ЛИШАЙ СКЛЕРОТИКОАТРОФИЧЕСКИЙ, ЛИШАЙ СКЛЕРОТИЧЕСКИЙ
Korean 경화위축성 태선
Polish Liszaj twardzinowy i zanikowy, Liszaj twardzinowy
Hungarian Lichen sclerosus és atrophicus, Fehér folt betegség, Lichen sclerosus, Lichen sclerosus et atrophicus, Lichen sclerosus et atrophichus