Assessment and management of cutaneous lupus erythematosus

In a patient with suspected cutaneous lupus erythematosus, assessment involves a full history and physical examination, together with appropriate laboratory investigations to determine the presence and extent of systemic disease (especially in patients with acute cutaneous lupus erythematosus). Skin biopsy for histology and direct immunofluorescence confirms the diagnosis of cutaneous lupus erythematosus.

Testing for antinuclear antibody (ANA), extractable nuclear antigen (ENA) antibody, double stranded DNA (dsDNA) antibody, and complement C3 and C4 levels is required to determine presence and extent of systemic disease. Patients with cutaneous lupus erythematosus are less likely to be ANA positive, compared to patients with systemic lupus erythematosus. Depending on the type of cutaneous lupus and presence of systemic involvement, ENA antibodies (including Ro and La subtype antibody) and dsDNA antibody may be positive. Complement C3 and C4 levels may be reduced in systemic lupus erythematosus. For more information, see Diagnosis of systemic lupus erythematosus in the Rheumatology guidelines.

If cutaneous lupus erythematosus (with or without systemic disease) is suspected, early referral to a dermatologist or rheumatologist (depending on whether cutaneous or systemic involvement is predominant) is recommended for confirmation of diagnosis and management. Most patients with acute cutaneous lupus erythematosus require systemic therapy from a specialist (see also Systemic lupus erythematosus in the Rheumatology guidelines).

Review and stop any potentially triggering drugs, if appropriate; see Presentation of cutaneous lupus erythematosus for possible drug triggers.

Advise patients on strict sun protection to prevent skin flare-ups that can trigger a systemic inflammatory cascade. Sun protection measures include wearing high collars, long-sleeves and broad-brimmed hats, and using broad-spectrum SPF 50+ sunscreen. Also advise patients to stop smoking because smoking can worsen disease severity and affect efficacy of hydroxychloroquine treatment (if this is being used).

For initial treatment of localised, thin or mild plaques in cutaneous lupus erythematosus, apply a potent corticosteroid. Use:

Under specialist guidance, potent topical corticosteroids can be used on the face for cutaneous lupus erythematosus.

For hypertrophic (thick) or resistant lesions, especially in chronic cutaneous (discoid) lupus erythematosus, apply the potent corticosteroid twice daily and use an occlusive film dressing.

If cutaneous lupus erythematosus lesions do not respond, use a more potent corticosteroid. Use:

If the response to topical corticosteroids is still inadequate, intralesional corticosteroid injections are the next line of therapy for localised lesions.

If the response of localised lesions to intralesional corticosteroid injections is still inadequate, or if the patient presents with extensive cutaneous disease, severe flares, or signs of scarring or alopecia, refer for specialist review. If interim treatment is needed, use:

Specialist treatment options include hydroxychloroquine, acitretin, immune response modifiers (eg sulfasalazine, dapsone, biologic agents) and immunosuppressants (eg azathioprine, methotrexate, mycophenolate mofetil).

Patients with cutaneous lupus erythematosus can subsequently develop systemic disease despite not initially presenting with systemic involvement. Monitor patients with chronic (discoid) and subacute cutaneous lupus erythematosus by performing 6- to 12-monthly investigations (or more often if clinically indicated [eg with a change in clinical features]). ANA titre and ENA positivity do not correlate with severity of disease activity and repeat ANA/ENA testing is not required once shown to be positive. Markers of systemic lupus disease activity are raised dsDNA titre, low C3 or C4, raised C-reactive protein (CRP), raised erythrocyte sedimentation rate (ESR), haematological derangement, and impaired kidney function (eg presence of urinary protein or glomerular red cells [level and number indicate severity]).