Lupus erythematosus (LE) is a multisystem autoimmune connective tissue disease that can have protean effects on the skin. In some patients only the skin is involved, while in others, life-threatening internal complications can arise, ranging from destruction of the kidneys to cerebritis. Among its most typical cutaneous manifestations are:
- Photosensitive and well-marginated thin plaques on the malar cheeks with fine desquamation with or without erosions, edema, and poikiloderma (acute systemic lupus erythematosus, SLE).
- Scaly papules and plaques associated with patulous follicles that heal predominantly with scarring and/or poikiloderma (discoid lupus erythematosus, DLE).
- Psoriasiform or annular plaques that heal predominantly without scarring (subacute cutaneous lupus erythematosus, SCLE; neonatal LE).
- Verrucous papules and plaques (hypertrophic lupus erythematosus).
- Non-scaly, infiltrative arcuate or serpiginous papules and plaques (tumid lupus erythematosus).
- Erythematous nodules that heal with deep atrophy (lupus erythematosus panniculitis).
- Vesicles or bullae (bullous lupus erythematosus).
Any mucosal surface may be affected by LE. Most forms of LE are mediated by lymphocytes, in a spectrum called “lupus dermatitis” by the late Dr. A. Bernard Ackerman, while bullous SLE, and some urticarial lesions are mediated by neutrophils. In the lupus anticoagulant syndrome, purpuric lesions occur with little or no inflammation.