Cutaneous Lupus Erythematosus

Aka: Cutaneous Lupus Erythematosus, Cutaneous Lupus, Discoid Lupus Erythematosus, Discoid Lupus, Chronic Cutaneous Lupus, Scarring Discoid Lupus, Subacute Cutaneous Lupus, Acute Cutaneous Lupus, CLE, CCLE, SCLE, ALE

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II. Classification: Chronic Cutaneous Lupus (CCLE)

  1. Discoid Lupus Erythematosus (DLE)
    1. Most common form of Chronic Cutaneous Lupus
    2. Peak Incidence in ages 30 to 40 years
    3. More common in women
    4. Exacerbated by Trauma and sunlight
    5. Asymmetric red 1-2 cm Plaques with adherent scale on the face and scalp, neck and ears
      1. Progresses to local skin atrophy over months
      2. Ultimately results in scar
        1. Scar with altered pigmentation (Hypopigmentation, Hyperpigmentation)
        2. Usually depressed scar, but may be hypertrophic
      3. Several presentation variants
        1. Generalized variant also involves trunk and extremities
        2. Hypertrophic variant
  2. Subacute Cutaneous Lupus (SCLE)
    1. Mid-category between CCLE and ACLE
    2. May be associated with Systemic Lupus Erythematosus or a drug-induced event
    3. Usually heals without scarring (unlike CCLE)
    4. Annular erythematous Plaques (psoriaform)
      1. Distributed over Shoulders and Forearms, neck and upper trunk
      2. Central Hypopigmentation and Telangiectasia
  3. Acute Cutaneous Lupus (ACLE)
    1. Occurs in combination with Systemic Lupus Erythematosus in most cases
    2. Malar Rash or Butterfly Rash (most common skin manifestation in ACLE)
      1. Erythema over the cheeks and bridge of nose
    3. Red Plaques
      1. Occurs on face, chest, and Shoulders
      2. Extensor arm and hand dorsum also involved
    4. Other skin manifestations
      1. Toxic Epidermal Necrolysis type skin eruption

III. Differential Diagnosis

  1. See Malar Rash
  2. See Annular Lesion
  3. Tinea Faciale
  4. Granuloma Faciale
  5. Sarcoidosis
  6. Cutaneous Leishmaniasis
  7. Cutaneous Tuberculosis
  8. Lymphoproliferative disorders (e.g. Lymphoma, Leukemia)

IV. Labs

  1. Skin Biopsy
    1. Consider ordering immunofluorescence for older lesion
  2. Serologic Testing if biopsy suggests Cutaneous Lupus
    1. ANA titer (positive if 1:160 or higher)
      1. Chronic Cutaneous Lupus (CCLE): 4%
      2. Subacute Cutaneous Lupus (SCLE): 63%
      3. Acute Cutaneous Lupus (ACLE): 98%
    2. Anti-dsDNA
      1. Subacute Cutaneous Lupus (SCLE): 30%
      2. Acute Cutaneous Lupus (ACLE): 60-80%
    3. Complement (C3 or CH50)
      1. Suppressed in 90% of Acute Cutaneous Lupus (ACLE)
    4. Anti-Ro Antibody (Anti-SSA)
      1. Associated with Subacute Cutaneous Lupus (SCLE)
  3. Further evaluation
    1. See Systemic Lupus Erythematosus
    2. Distinguish systemic from isolated skin involvement

V. Management

  1. Sunscreen with UVA and UVB protection is critical
  2. Non-systemic agents
    1. Topical Corticosteroids (first choice)
    2. Calcineurin Inhibitors (e.g. Tacrilimus)
    3. Physical treatments
      1. Laser therapy
      2. Cryotherapy
      3. Dermabrasion
    4. Intralesional Corticosteroid
      1. Indicated for Discoid Lupus (DLE, CCLE)
  3. Systemic agents
    1. First-line: Hydroxychloroquine (Plaquenil)
    2. Other agents (if refractory to topicals, Plaquenil)
      1. Dapsone
      2. Oral Corticosteroids
      3. Azathioprine
      4. Isotretinoin
      5. Methotrexate

VI. References

  1. Merola (2019) UpToDate, accessed 12/4/2019
  2. Habif (1996) Dermatology, p. 544-9
  3. Okon (2013) Best Pract Res Clin Rheumatol 27(3): 391–404. [PubMed]
  4. Patel (2002) Dermatol Clin 20(3):373-85 [PubMed]

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