Lupus erythematosus

Last Updated: 2025-07-14

Author(s): Anzengruber F., Navarini A.

ICD11: 4A40.Z

  • Cazenave and Schedel, 1833
  • Kaposi, 1872
  • Leloir, 1890

Lupus erythematosus, LE, erythematodes, butterfly lichen, Leloir's disease

Chronic autoimmune disease of the skin and/or internal organs, mediated by loss of immune tolerance, autoantibody production, and interferon-driven inflammation.

  • Peak onset: 20th to 40th year of life
  • Female : male = approx. 9:1 (in SLE)
  • Cutaneous lupus erythematosus (CLE) is 2–3× more common than SLE
  • Higher prevalence in individuals with skin of colour
  • Worldwide SLE prevalence: 20–150/100,000

Systemic lupus erythematodes (SLE)


Cutaneous lupus erythematosus (CLE)

 

  • Acute cutaneous lupus erythematosus (ACLE)
    • Localised ACLE
    • Generalised ACLE
  • Subacute cutaneous lupus erythematosus (SCLE)
    • Annular SCLE
    • Papulosquamous SCLE
  • Chronic cutaneous lupus erythematosus (CCLE)
    • Discoid lupus erythematosus (DLE)
      • Localised DLE
      • Generalised DLE
    • Mucosal DLE (oral/conjunctival)
    • Lupus erythematosus verrucosus (hypertrophicus)
    • Lupus erythematosus profundus (panniculitis)
  • Intermittent CLE
    • Lupus erythematosus tumidus (LET)

 

Special forms:

  • Rowell's syndrome
  • Chilblain lupus
  • Drug-induced lupus erythematosus
     

  • Genetic predisposition (HLA-DR2/DR3, IRF5, STAT4, TYK2, PTPN22)
  • Breakdown of immune tolerance
  • Pathogenic autoantibodies: ANA, anti-dsDNA, anti-Sm, anti-Ro/SSA, anti-La/SSB
  • Type I interferon axis activation (IFN-α, IFNAR1 signaling)
  • Dysregulated NETosis, oxidative stress
  • Environmental triggers: UV radiation, smoking, viral infections, drugs (e.g. hydralazine, procainamide)
     

  • ACLE: malar (butterfly) erythema, widespread photosensitive rash
  • SCLE: annular or psoriasiform plaques, symmetrical on sun-exposed areas
  • DLE: erythematous plaques with scale, follicular plugging, scarring and pigment changes
  • LET: smooth, erythematous, oedematous plaques without surface changes
  • Mucosal LE: oral ulcers, rarely conjunctival involvement
  • Nailfold signs: periungual telangiectasias, nail dystrophy
     

  • Classification of SLE should follow the 2019 EULAR/ACR criteria, which require a positive ANA (≥1:80 on HEp-2 cells) as the entry criterion, followed by additive weighted clinical and immunological criteria. A total score of ≥10 points confirms the diagnosis of SLE.
  • ANA testing is mandatory because it is the required entry criterion in the 2019 classification. ANA positivity reflects underlying B-cell activation and serves as a highly sensitive (but non-specific) marker of systemic autoimmunity. In CLE, ANA can also support the diagnosis and help identify patients at risk for systemic progression.
  • Clinical diagnosis supported by laboratory and histology
  • Serology: ANA, anti-dsDNA, anti-Sm, anti-Ro/SSA, anti-La/SSB
  • Complement (C3/C4), ESR/CRP, urinalysis
  • Skin biopsy: interface dermatitis, lymphocytic infiltrates, basement membrane thickening
  • Direct immunofluorescence (DIF): linear IgG, C3 at dermoepidermal junction (Lupus band test)
  • CLASI (Cutaneous Lupus Erythematosus Disease Area and Severity Index) for scoring
     

  • ACLE: face (malar), upper trunk, arms
  • SCLE: upper chest, upper back, shoulders, arms
  • DLE: scalp, face, ears; can cause scarring alopecia
  • LET: face, neck, upper torso
  • Lupus profundus: subcutis of upper arms, thighs, buttocks
  • Chilblain LE: acral areas during cold exposure
     

  • Fluctuating course with environmental (e.g. UV light) or hormonal triggers
  • Family history of autoimmune disease common

  • Interface dermatitis with basal vacuolization
  • Follicular plugging, hyperkeratosis
  • Dermal mucin deposition
  • Periadnexal and perivascular lymphocytic infiltrates
  • DIF: positive lupus band test (IgG/C3 along basement membrane)
     

  • DLE: scarring, dyspigmentation, secondary infection
  • Risk of squamous cell carcinoma in long-standing DLE lesions
  • SCLE: ~15–20% transition to systemic lupus
  • Systemic complications in SLE: lupus nephritis, CNS involvement, cytopenias, serositis
     

  • Strict photoprotection (broad-spectrum sunscreen ≥ SPF 50)
  • Smoking cessation
  • Screening for and managing comorbid autoimmune diseases
  • Vitamin D supplementation as needed
     

  • Variable depending on subtype
  • LET: good prognosis, no scarring
  • DLE: potential for permanent scarring
  • SCLE: relapsing, potential SLE progression
  • SLE: prognosis determined by organ involvement (e.g. renal, CNS)
     

  1. Fanouriakis A et al. 2023 EULAR recommendations for the management of systemic lupus erythematosus. Ann Rheum Dis. 2023;82:15–27.
  2. Kuhn A et al. Updated classification and pathogenesis of cutaneous lupus erythematosus. J Eur Acad Dermatol Venereol. 2022;36(8):1214–1224.
  3. Stannard J et al. Anifrolumab in moderate to severe cutaneous lupus erythematosus: a phase 3 trial. N Engl J Med. 2023;388(9):841–851.
  4. Durosinmi-Etti F et al. Cutaneous lupus erythematosus: diagnostic and therapeutic updates. JAMA Dermatol. 2024;160(2):125–134.
  5. Swissmedic. Saphnelo® Zulassung für systemischen Lupus erythematodes. Swissmedic Bulletin. 2023.