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Lichen Sclerosus

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Inflammatory disease primarily of the superficial dermis or submucosa that leads to ivory-white scar-like atrophy.
 
Epidemiology
  • unknown
  • female to male ratio varies from 10:1 to 1:1
  • Anogenital area affected in 85% of patients
  • Lichen sclerosus diagnosed in 13-19% of patients presenting with symptomatic vulvar disease → DDx not to forget in STDs
  • Bimodal distribution
    • Prepubertal
    • Perimenopausal
 
Etiology and mechanism: auto-immune
  • Antibodies against Extracellular matrix protein 1 (ECM-1) (present in 80% of patients)
    • ECM-1 is a secreted glycoprotein of the extracellular matrix
 
Clinical presentation
  • Women
    • Vulva and perianal area in figure of 8
    • Fusion of labia minora and majora
    • Sexual intercourse may become impossible
    • Follicular plugging
    • Hemorrhagic bullae
  • Men
    • Phimosis
    • Recurrent balanitis
    • Perianal involvement is rare
    • Itching and soreness
  • Increased risk of malignancy (CEC) - controversy if LS is precancerous or not
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Histologia
  • Padrão em bandeira com 3 partes
    • Camada cornea e epiderme atrófica (infiltrado em banda na junção nas fases iniciais)
    • Zona de esclerose - banda de esclerose que “empurra” o infiltrado para baixo
    • Infiltrado por baixo da esclerose
  • Espessamento de hialina da membrana basal (típico do líquen escleroso e lúpus)
  • Padrão de dermatite interface
  • Esclerose da derme - hialina mais espessada, hialinização do tecido conjuntivo, aspeto mais hialino da derme papilar
 
Abordagem diagnóstica:
  • Abordagem: Biópsia logo e depois tratar
  • Abordagem: tratar com dermovate 10 semanas, se não resolver fazer biópsia
 
DDx
  • Vitiligo VS Lichen sclerosus
    • Vitiligo is asymptomatic. Lichen sclerosus usually has pain or pruritus or burning
    • Vitiligo has sharp demarcated borders
    • Lichen sclerosus is ivory white macules
    • Vascular lesions: ecchymosis, purpura, telanciectasia, angiokeratoma sugere inflamação in lichen sclerosus
  • Morphea
  • Sexual abuse
 
Tratamento
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Gets better after puberty. But is it absense of symptoms or absense of disease?
  • Treatment considerations
    • Early treatment and ongoing management is recommended
      • Goal: to relieve symptoms and prevent scarring, adhesions and SCC
    • No risk of squamous cell carcinoma in children but are cases of scc in adults with infantile onset lichen sclerosus
    • Risk of permanent scarring
  • Emollients
  • Ultrapotent corticosteroids (dermovate) for 3 months
  • Combination of corticosteroids and calcipotriol reduces the risk of skin atrophy
  • calcineurin inhibitors 2nd line if topical corticosteroids are contraindicated
  • maintenance therapy?
  • Plaquinol
  • Dermovate → protopic
  • Circuncisão pode ser necessário
  • UVA 1 Phototherapy
  • Photodynamic therapy
  • Failure of therapy
    • Consider candidiasis or menopausal vulvo-vaginal atrophy
  • If systemic widespread disease
    • Weekly methotrexate 10-20mg
    • Systemic corticosteroids
  • Em investigação
    • Ruxolitinib (JAK inhibitor)