Table of contents
Cutaneous lupus erythematosus
Guidelines
Key sources
The following summarized guidelines for the evaluation and management of cutaneous lupus erythematosus are prepared by our editorial team based on guidelines from the European League Against Rheumatism (EULAR 2024,2023), the American College of Rheumatology (ACR 2023), the British Association of Dermatologists (BAD 2021), the Chinese Society of Dermatology (CSD/ADA/AADV 2021), the German Society of Dermatology (DDG/GKJR/DGRh 2021), the Royal ...
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Screening and diagnosis
Diagnostic criteria
As per DDG/DGRh/GKJR 2021 guidelines:
Use the 2019 EULAR/ACR criteria to differentiate CLE from SLE.
A
Consider reassessing the 2019 EULAR/ACR criteria either once a year or in case of clinical/laboratory changes in any patient with CLE.
C
EULAR/ACR classification criteria for systemic lupus erythematosus
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Entry criteria
Anti-nuclear antibodies at a titer of ≥ 1:80 on HEp-2 cells or an equivalent positive test ever)
No positive test
Constitutional symptoms
Fever
Hematologic features
Leukopenia
Thrombocytopenia
Autoimmune hemolysis
Neuropsychiatric presentations
Delirium
Psychosis
Seizure
Mucocutaneous involvement
Non-scarring alopecia
Oral ulcers
Subacute cutaneous or discoid lupus
Acute cutaneous lupus
Serosal involvement
Pleural or pericardial effusion
Acute pericarditis
Musculoskeletal involvement
Joint involvement
Renal involvement
Proteinuria > 0.5 g/24 hours
Renal biopsy Class II-V lupus nephritis
Renal biopsy Class III-IV lupus nephritis
Antiphospholipid antibodies (anti-cardiolipin antibodies, anti-β2GP1 antibodies, or lupus anticoagulant)
Present
Absent
Complement proteins
Low C3 or C4
Low C3 and C4
Systemic lupus erythematosus-specific antibodies (anti-double stranded DNA antibody or anti-Smith antibodies)
Present
Absent
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Differential diagnosis
Classification and risk stratification
Severity assessment
As per DDG/DGRh/GKJR 2021 guidelines:
Consider using the CLASI or revised CLASI to assess disease activity and intensity in patients with CLE.
C
Consider using the Dermatology Life Quality Index or Skindex-29 (skin-specific methods for assessing the QoL) to assess the QoL in patients with CLE.
C
Diagnostic investigations
History and physical examination: as per BAD 2021 guidelines, evaluate for SLE by history, examination and targeted laboratory investigations in patients with CLE.
B
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Photoprovocation testing
Laboratory testing
Pretreatment evaluation
Diagnostic procedures
Medical management
General principles: as per BAD 2021 guidelines, manage patients with CLE in the setting of SLE jointly with a rheumatologist.
E
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Topical therapy
Antimalarial agents
Systemic corticosteroids
Methotrexate
Systemic retinoids
Dapsone
Mycophenolate mofetil
Other immunosuppressants
Thalidomide and lenalidomide
Monoclonal antibodies
Other agents
Nonpharmacologic interventions
Lifestyle modifications: as per BAD 2021 guidelines, counsel patients with CLE about the importance of lifestyle changes on disease activity and treatment response, such as smoking cessation and the need for a range of photoprotective measures, including the use of a broad-spectrum sunscreen.
A
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Vitamin D supplementation
Herbal products
Psychological support
Therapeutic procedures
Intralesional corticosteroids: as per BAD 2021 guidelines, consider administering intralesional triamcinolone (0.1 mL/cm² field, starting at 2.5-5 mg/mL for sites at higher risk of atrophy including the face and 10 mg/mL for other sites) as a local treatment option in patients with localized DLE or as an adjunctive therapy for persistent lesions.
E
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Physical modalities
Specific circumstances
Considerations for contraception: as per DDG/DGRh/GKJR 2021 guidelines, consider preferring non-hormonal contraceptives or progestin-only contraceptives.
C
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Patients contemplating pregnancy
Pregnant patients
Patient education
General counseling
As per BAD 2021 guidelines:
Offer obtaining a pregnancy test in female patients of child-bearing potential before initiating methotrexate, mycophenolate mofetil, acitretin, rituximab, belimumab, cyclophosphamide, thalidomide or lenalidomide therapy. Counsel them regarding the risk of teratogenicity, advise pregnancy prevention and instigate the pregnancy prevention program.
A
Advise using reliable contraception as a precautionary measure in sexually active male patients during methotrexate, mycophenolate mofetil, thalidomide and lenalidomide therapy, during dose interruption (where applicable) and for periods of time following the cessation of treatment.
A
Preventative measures
Routine immunizations: as per ACR 2023 guidelines, consider offering high-dose or adjuvanted influenza vaccination, rather than regular-dose influenza vaccination, in ≥ 65 years old patients with rheumatic or musculoskeletal diseases and in 18-65 years old patients with rheumatic or musculoskeletal diseases on immunosuppressive medications.
C
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Prophylaxis for P. jirovecii pneumonia