Hydroxychloroquine is first-line systemic treatment for patients with chronic cutaneous lupus erythematosus, such as discoid lupus. The cutaneous manifestations of lupus may present focally, as with the classic “butterfly” malar rash, or with more diffuse findings. A unique form of lupus-associated skin disease is discoid lupus. Discoid lupus may occur in patients with clearly defined lupus but may also present in other patients with few other clinical manifestations of lupus. This patient has had chronic lesions for 3 years limited to a relatively small area on the head and neck. She lacks other symptoms of systemic lupus erythematosus (SLE). Low titer antinuclear antibody positivity may occur in patients with skin-limited chronic cutaneous lupus. Topical therapy with glucocorticoids and calcineurin inhibitors is the usual initial treatment approach. For patients who fail to improve, systemic antimalarial therapy such as hydroxychloroquine, which is the systemic treatment of choice for most manifestations of cutaneous lupus, is indicated. There is some evidence that treatment with hydroxychloroquine can diminish the risk of progression to overt SLE in some patients.

Dapsone is a sulfonamide that has efficacy in treating specific types of lupus-associated skin disease. However, along with methotrexate, mycophenolate mofetil, and systemic retinoids, dapsone is considered second-line therapy for cutaneous lupus.

Although systemic glucocorticoids such as prednisone are sometimes used in systemic lupus, this patient does not have SLE (negative titers for anti-Smith and antidouble-stranded DNA, normal complete blood count and urinalysis, and no extracutaneous signs or symptoms). Because of this and the potential associated adverse effects of systemic glucocorticoids, they are not routinely used in the management of lupus skin disease.

Vitamin D supplementation, even in high dosages, has not shown efficacy in preventing or treating the cutaneous manifestations of lupus.