Cutaneous lupus erythematosus (LE) may present in a variety of clinical forms. Three recognized subtypes of cutaneous LE are acute cutaneous LE (ACLE), subacute cutaneous LE (SCLE), and chronic cutaneous LE (CCLE). ACLE may be localized (most often as a malar or '
butterfly' rash) or generalized. Multisystem involvement as a component of systemic LE (SLE) is common, with prominent musculoskeletal symptoms. SCLE is highly photosensitive, with predominant distribution on the upper back, shoulders, neck, and anterior chest. SCLE is frequently associated with positive anti-
Ro antibodies and may be induced by a variety of medications. Classic discoid LE is the most common form of CCLE, with indurated scaly plaques on the scalp, face, and ears, with characteristic
scarring and pigmentary change. Less common forms of CCLE include hyperkeratotic LE, lupus tumidus,
lupus profundus, and
chilblain lupus. Common cutaneous disease associated with, but not specific for, LE includes
vasculitis, livedo reticularis,
alopecia, digital manifestations such as periungual
telangiectasia and
Raynaud phenomenon, photosensitivity, and
bullous lesions. The clinical presentation of each of these forms, their diagnosis, and the inter-relationships between cutaneous LE and SLE are discussed. Common systemic findings in SLE are reviewed, as are diagnostic strategies, including histopathology, immunopathology, serology, and other laboratory findings. Treatments for cutaneous LE initially include preventive (e.g. photoprotective) strategies and topical
therapies (
corticosteroids and topical
calcineurin inhibitors). For
skin disease not controlled with these interventions, oral
antimalarial agents (most commonly
hydroxychloroquine) are often beneficial. Additional systemic
therapies may be subdivided into conventional treatments (including
corticosteroids,
methotrexate,
thalidomide,
retinoids,
dapsone, and
azathioprine) and newer
immunomodulatory therapies (including
efalizumab, anti-
tumor necrosis factor agents,
intravenous immunoglobulin, and
rituximab). We review evidence for the use of these medications in the treatment of cutaneous LE.