Subacute CLE (SCLE)

  • ~50% meet SLICC criteria, 10-15% have severe SLE
  • >1/3 are drug-induced (drug discontinuation may or may not decrease lesions)

  • Photodistributed on upper trunk and extensors of upper extremities. Usually relative sparing of face.
  • Maculopapular with variable scale and dyspigmentation, usually no scarring

  • Annular SCLE
  • Papulosquamous SCLE

  • Epidermal atrophy, vacuolar alteration of basal layer, apoptotic keratinocytes, follicular plugging, basement membrane thickening
  • Lymphocytic infiltrate in upper dermis + periadnexal
  • ± Hyperkeratosis, dermal edema, dermal mucin

  • Papulosquamous (psoriasis, tinea)
  • Photolichenoid drug eruption
  • Polymorphous light eruption
  • Granuloma annulare
  • Figurate erythemas (e.g. erythema annulare centrifugum)
  • Dermatitis – atopic, contact and photocontact
  • Pemphigus foliaceus
  • Mothers of children with X-linked chronic granulomatous disease and individuals with Sjögren syndrome have comparable annular erythema

  • Infant of mothers with anti-Ro+ > La+ SCLE, Sjögren, or undifferentiated autoimmune syndrome are at risk
    • 50% of mothers are asymptomatic but may develop one of the conditions within ~3 years
    • 10-20% of women with SCLE will have a baby with NLE
    • Mothers with 1 child with NLE have 30% risk of their other children having NLE
  • SCLE-like lesions (50-80%)
    • Predilection to face (periorbital & scalp) “raccoon eyes / owl eyes”
    • May be photosensitive, but lesions may occur without sun exposure (present at birth or soon after)
    • Histologically similar to SCLE
  • Internal manifestations:
    1. Congenital heart block (10-30%) ± cardiomyopathy; 2/3 need pacemaker, 20% mortality
    2. Hepatobiliary disease (10-30%) – at birth to first few months
      • Liver failure with conjugated hyperbilirubinemia
      • Mild elevation aminotransferases at 2-3 months
    3. Cytopenias (esp. thrombocytopenia) – at birth or first months
  • Reports of hydrocephalus, microangiopathic hemolysis, and disseminated intravascular coagulation
  • Systemic evaluation recommended until 6-9 months:
    • History/physical exam: growth, head circumference
    • ECG ± echo, CBC, LFTs, creatinine, ANA, ENA especially Ro/SSA and La, C3, C4, Coombs test (lupus anticoagulant), urinalysis. Repeat every 2–3 months
    • Liver ultrasound
    • Family counselling
  • Treatment: Photoprotection, topical steroid s /calcineurin inhibitors, systemic steroids, IVIG.
  • Pre-emptive treatment: mothers of infants with cardiac NLE→ hydroxychloroquine for subsequent pregnancies and weekly fetal cardiac echo between 18-26 weeks of gestation