Lupus Erythematosus, Discoid

Basics

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)

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