lichen sclerosus et atrophicus (LSA)

Introduction

Chronic atrophic mucocutaneous disorder characterized by white, angular, well-delineated, indurated papules & plaques.

Etiology

unknown

Epidemiology

• adults & children 1-13 years of age
• mean age of onset: 43 in males, 50 in females
• female:male ratio 10:1

Pathology

• epidermis:
  • variably thickened, hyperkeratotic with follicular plugging (early)
  • atrophic (late)
• dermis
  • band of homogeneous dermal collagen below epidermis
  • structureless & edematous
  • lymphocytic infiltrate
    • subepidermal (early)
    • below structureless & edematous dermis (later)
  • dilated capillaries & hemorrhage

* histopathology images

Clinical manifestations

• lesions may be present for 1 year prior to detection
• generally asymptomatic
• in females vulvar lesions may be sensitive, especially while walking, or may be painful if erosions are present
• in males, phimosis, recurrent balanitis
• macules & papules, whitish in color, sharply demarcated
• individual lesions may become confluent forming plaques
• lesions may be elevated, flat or older lesions may be depressed
• puncta in center
• bullae & erosions may heal with fusion of the labia minora
• purpura
• telangiectasia
• keratotic plaques on vulva may become macerated
• vulva may become atrophic
• distribution:
  • genital:
    • vulva in females
    • prepuce or glans penis in males
  • non-genital: trunk, esp upper back, periumbilical region, neck, axillae, flexor surface of wrists, rarely palms& soles
  • oral mucosa: blueish-white plaques on buccal or palatal mucosa &/or tongue
• disease waxes & wanes

* images

Complications

• squamous cell carcinoma, predominantly in males (uncommon)

Differential diagnosis

• morphea (may coexist)
• lichen simplex chronicus
• lupus erythematosus
• leukoplakia
• lichen planus
• intra-epithelial neoplasia (Bowen's disease)
• extramammary Paget's disease
• intertrigo
• candidiasis

Management

• symptomatic: no curative therapy
• topical corticosteroids
  • potent steroids effective for genital LSA
  • may be mixed with topical androgens
• systemic therapy: hydroxychloroquine 250 mg PO QD
• circumcision relieve phimosis in males

More general terms

• lichen sclerosus