Approach Considerations
The goals of management in subacute cutaneous lupus erythematosus (SCLE) are to improve the patient's lesions, mitigate any associated symptoms, prevent the development of additional lesions, and assess for potential associated systemic disease. [28] Counseling patients regarding the risk of serious systemic disease is important. Although many patients fulfill the criteria for systemic lupus erythematosus (SLE), the severity of systemic manifestations tends to be more mild, and renal and central nervous system disease occur less frequently than in patients with SLE without associated SCLE.
Surgical approaches rarely are needed in patients with SCLE. Cosmetic measures are often less important in patients with this condition than in patients with discoid lupus erythematosus (DLE), given the lack of associated scarring; however, active disease and postinflammatory dyspigmentation may still cause notable cosmetic concerns in patients with SCLE. No special diet is required with SCLE.
Inpatient care
Inpatient care is rarely needed for patients with skin disease; however, since these patients may have SLE, they may occasionally manifest internal complications that require hospitalization. In these instances, consultation with other physicians may be helpful.
Skin Protection
Therapy begins with sun-protective measures, including sunscreens, protective clothing, and behavior alteration. A sunscreen containing Mexoryl SX and Mexoryl XL, tested in a randomized, placebo-controlled trial, was demonstrated to prevent the development of ultraviolet-induced cutaneous lesions. [29, 30]
Another study demonstrated that in an experimental setting, cutaneous lupus erythematosus (CLE) can be prevented by the use of a particular broad-spectrum sunscreen. The use of sunscreens in a clinical setting is not as effective as would be predicted from studies such as this one, perhaps because patients do not perform sunscreen application on a daily basis in a manner that might be effective. [31]
Broad-spectrum sunscreen with a sun protection factor of 50 or greater should be applied daily, at least 20 minutes prior to sun exposure, and should be reapplied every 2-3 hours. Sun avoidance and sun-protective clothing are necessary in patients with subcutaneous lupus erythematosus (SCLE). Of note, skin disease in SCLE is often very challenging to control without adequate photoprotection.
Pharmacologic Therapy
Standard therapy for subacute cutaneous lupus erythematosus (SCLE) includes topical corticosteroids and antimalarials such as hydroxychloroquine or chloroquine. In patients with skin lesions refractory to hydroxychloroquine monotherapy, adding quinacrine can improve response. [32] A French multicenter study found that the measurement of hydroxychloroquine blood levels may correlate with the incidence of remission in patients with cutaneous lupus erythematosus (CLE). Another finding of this study is that blood levels can demonstrate a lack of compliance with therapy; those patients with very low levels had little clinical response. [33]
Additional therapies that may be considered in selected patients include thalidomide, methotrexate, mycophenolate mofetil, azathioprine, retinoids, dapsone, intravenous immunoglobulin, cyclosporine, auranofin, clofazimine, interferon, immunosuppressive agents such as cyclophosphamide and rituximab, and the monoclonal antibody belimumab. [34, 35, 36, 37]
Patients who smoke appear to respond less well to antimalarial therapy.
Lenalidomide, a thalidomide analogue, can be helpful in treating patients with refractory CLE as well. Studies have shown skin improvement measured by CLASI (Cutaneous Lupus Erythematosus Disease Area and Severity Index) scores as early as 2 weeks after treatment initiation, although clinical relapse was common following medication withdrawal. [38]
Avoid systemic corticosteroids except for acute short-term usage or when the presence of systemic disease warrants use.
Some patients with CLE have been found to be vitamin D deficient, particularly those who use careful photoprotection; therefore, the use of vitamin D and calcium should be considered following an assessment of vitamin D levels. [39]
Several upcoming therapeutic targets may lead to additional treatment options for patients with cutaneous lupus in the future. [40]
Consultations
Consultations with the following specialists may be helpful:
-
Rheumatologist - When joints and/or systemic involvement are involved
-
Nephrologist - When renal involvement is present
-
Neurologist - When central nervous system disease is present
-
Hematologist – When cytopenias are present
-
Ophthalmologist – Patients treated with antimalarials need at least annual ocular evaluations
Long-Term Monitoring
Follow patients with subacute cutaneous lupus erythematosus (SCLE) at regular intervals. Response to therapy varies, depending on the therapeutic agent prescribed. Avoid changes in therapy until the typical period for medication maximal efficacy elapses.
At least once each year, and perhaps twice, assess stable patients using routine laboratory tests, including complete blood cell count, renal function tests, and urinalysis. Repeat autoantibody testing is of little use in patients with SCLE, unless they have systemic lupus erythematosus (SLE). Regularly assess historical information concerning additional systemic manifestations of SLE.
-
Early lesions of subacute cutaneous lupus erythematosus may simulate polymorphous light eruption.
-
Papulosquamous lesions of subacute cutaneous lupus erythematosus may simulate psoriasis.
-
Annular lesions of subacute cutaneous lupus erythematosus.
-
Tumid lupus erythematosus.
-
Neonatal lupus erythematosus.
