Description

• Discoid lupus erythematosus (DLE) is the most common form of cutaneous LE.
• DLE is an inflammatory condition that can lead to permanent scarring, hair loss, and disfigurement.
• DLE is triggered by sun exposure and typically presents as well-demarcated, erythematous, indurated, and scaly plaques.
• DLE types:
  • Localized DLE
    • Occurs most often on face, neck, and scalp
  • Disseminated/generalized DLE
    • Occurs on areas of the body below the neck
• Synonym(s): chronic cutaneous LE; subacute cutaneous LE; discoid lupus

Pediatric Considerations
Neonatal LE is a rare autoimmune disorder (caused by transplacental passage of maternal antibodies) that presents with cutaneous lupus skin lesions and/or other systemic manifestations, including congenital heart block.

Epidemiology

• DLE occurs in persons of all ages and ethnicities.
• All forms of cutaneous LE are most common among women of childbearing age.

Incidence
DLE manifests in up to 25% of patients with systemic lupus erythematosus (SLE) but can occur without systemic involvement:

• Predominant age: 21 to 50 years
• Predominant sex:
  • Localized DLE: female > male (3:1)
  • Generalized DLE: female > male (9:1)

Prevalence

• Prevalence of cutaneous LE: 73/100,000
• Increased prevalence in women and also in persons of African descent
• Between the ages of 21 and 30 years, prevalence is similar among both sexes.

Etiology and Pathophysiology

• DLE is an autoimmune dysfunction of T cells linked to human leukocyte antigen (HLA) subtypes and environmental triggers.
• Keratinocyte heat shock protein induction after UV light exposure or stress as a target for T-cell–mediated epidermal cytotoxicity

Genetics

• DLE may occur in genetically predisposed.
• A haplotype of cytotoxic T-lymphocyte–associated protein 4 (CTLA4) showed association with DLE.

Risk Factors

• Adult women of childbearing age
• African American
• SLE

General Prevention

• Avoid exposure to UV-B and UV-A light sources.
• Excessive heat, excessive cold, and trauma to the affected skin may worsen DLE.
• Use appropriate sunscreen, protective clothing (dark colors and closely woven fabrics), and hats.
• Tobacco exposure may decrease antimalarials efficacy.

Commonly Associated Conditions

• SLE
• Mixed connective tissue disease
• Antiphospholipid syndrome
• Porphyria cutanea tarda (PCT)