RT Book, Section A1 Sontheimer, Clayton J. A1 Costner, Melissa I. A1 Sontheimer, Richard D. A2 Kang, Sewon A2 Amagai, Masayuki A2 Bruckner, Anna L. A2 Enk, Alexander H. A2 Margolis, David J. A2 McMichael, Amy J. A2 Orringer, Jeffrey S. SR Print(0) ID 1161332248 T1 Lupus Erythematosus T2 Fitzpatrick's Dermatology, 9e YR 2019 FD 2019 PB McGraw-Hill Education PP New York, NY SN 9780071837798 LK accessmedicine.mhmedical.com/content.aspx?aid=1161332248 RD 2024/04/19 AB AT-A-GLANCEA group of heterogeneous illnesses that have in common the development of immunity to self-nucleic acids and their associated proteins, with skin-only disease at one end of the spectrum and severe visceral involvement at the other.Skin lesions may be specific to lupus or nonspecific, being seen in other conditions as well.Acute cutaneous lupus erythematosus (malar rash) is almost always associated with underlying visceral involvement. Subacute cutaneous lupus patients meet American College of Rheumatology systemic lupus erythematosus criteria approximately 50% of the time (but typically express only mild systemic clinical manifestations). Chronic cutaneous lupus (classic discoid lupus erythematosus, lupus panniculitis, chilblain lupus, and tumid lupus erythematosus) patients most often have skin-only or skin-predominant disease.Classical discoid lupus erythematosus causes scarring and can be permanently disfiguring. Subacute cutaneous lupus and acute cutaneous lupus erythematosus are highly photosensitive and are characteristically nonscarring.Lupus erythematosus–nonspecific skin lesions include nonscarring alopecia, mouth ulcers, photosensitivity, Raynaud phenomenon, vasculitis/vasculopathy, and bullous systemic lupus erythematosus, among others. They often herald a systemic lupus erythematosus flare.Treatment consists of physical sun protection, topical sunscreens, local and short-term systemic glucocorticoids, antimalarials, retinoids, thalidomide/lenalidomide, conventional immunosuppressives, and biologic therapies.Lupus erythematosus occurs much more commonly in women (9:1 female-to-male ratio).Both systemic lupus erythematosus and cutaneous lupus erythematosus are associated with upregulation of type 1 interferon signaling.