Discoid lupus erythematosus (DLE) is a disfiguring autoimmune skin disease and the most common form of chronic cutaneous lupus erythematosus (CLE). DLE most commonly affects women in the second, third, and fourth decades of life, although it may occur at any age. Black patients with DLE may be more at risk for severe disease, and there may be a positive family history of systemic lupus erythematosus (SLE) or other connective tissue disease.

Discoid rash is one of 11 clinical criteria for the Systemic Lupus International Collaborating Centers (SLICC) classification of SLE and is a contributor to the musculoskeletal clinical domain within the European League Against Rheumatism / American College of Rheumatology (EULAR / ACR) classification criteria for SLE.

DLE may be localized or generalized. Localized DLE is limited to the face, scalp, ears, and neck, while in generalized DLE, there may be lesions both above and below the neck. It is rare for DLE to be isolated below the neck. There is a 20% likelihood of SLE with generalized DLE and 5% with localized DLE. Conversely, approximately 15%-30% of patients with SLE will manifest discoid lesions. Other risk factors for SLE include mucocutaneus involvement, arthralgias ⁄ arthritis, nail changes, anemia, leukopenia, an elevated ESR, and a positive test for antinuclear antibodies (ANA).

Lower eyelid involvement occurs in 6% of discoid lupus patients and is likely triggered by sun exposure. Eyelid involvement may be associated with chronic blepharitis. Upper eyelid involvement may also be seen but is not as common. DLE-associated stromal keratitis has been reported. Periorbital mucinosis of DLE may also present as periorbital edema and erythema.

Squamous cell carcinoma may rarely develop in chronic DLE scars, especially in sun-exposed areas.

Related topics: drug-induced lupus erythematosus, lupus panniculitis, subacute cutaneous lupus erythematosus, tumid lupus erythematosus