Cutaneous lupus erythematosus - Diagnosis and management

Cutaneous lupus erythematosus (CLE) includes a variety of lupus erythematosus (LE)-specific skin lesions that are subdivided into three categories - chronic CLE (CCLE), subacute CLE (SCLE) and acute CLE (ACLE) - based on clinical morphology, average duration of skin lesions and routine histopathologic examination. This paper describes our personal experience in the management of CLE over the last 30 years, with details on preferential therapeutic options related to clinical, histologic and immunopathologic aspects of each clinical subset of the disease. Effective sunscreening and sun protection are considered the first rule in the management of CLE because of the high degree of photosensitivity of the disease. Antimalarial agents are crucial in the treatment of CLE and are the first-line systemic agents, particularly in discoid LE (DLE) and SCLE. Dapsone is the drug of choice for bullous systemic LE (BSLE) as well as for LE in small dermal vessels (e.g. leukocytoclastic vasculitis). Retinoids, known as second-line drugs for systemic therapy, are sometimes used to treat chronic forms of CLE and are particularly successful in treating hypertrophic LE. Systemic immunosuppressive agents are required to manage the underlying systemic LE disease activity in patients with ACLE. These drugs, especially azathioprine, methotrexate, cyclophosphamide and cyclosporine, together with corticosteroids, constitute third-line systemic therapy of CLE. In our experience, oral prednisone or parenteral 'pulsed' methylprednisolone are useful in exacerbations of disease activity. Thalidomide provides one of the most useful therapeutic alternatives for chronic refractory DLE, although its distribution is limited to a few countries because of the risk of teratogenicity and polyneuropathy. However, medical treatment with local corticosteroids remains the mainstay of CLE treatment, especially for DLE. Patient education regarding the disease is also important in the management of CLE, because it helps relieve undue anxiety and to recruit the patient as an active participant in the treatment regimen.