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Cutaneous lupus erythematosus

What's new

Updated 2023 EULAR guidelines for the management of 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 and/or in case of clinical/laboratory changes in any patient with CLE.
C
EULAR/ACR classification criteria for systemic lupus erythematosus
Entry criteria
ANA 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
SLE-specific antibodies (anti-dsDNA 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 DLQI 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

Biopsy and histopathology: as per DDG/DGRh/GKJR 2021 guidelines, perform lesional biopsy for histological confirmation of a clinical diagnosis of CLE, while omitting in cases of butterfly rash or mucosal lesions.
A
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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

Follow-up and surveillance

Assessment of treatment response: as per DDG/DGRh/GKJR 2021 guidelines, consider using the CLASI or the revised CLASI for monitoring therapeutic response.
C

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  • Follow-up

  • Surveillance for hydroxychloroquine retinopathy

  • Surveillance for malignancy