Discoid lupus erythematosus (DLE) is a disfiguring autoimmune skin disease and the most common form of chronic cutaneous lupus erythematosus. It has a characteristic clinical appearance consisting of red, scaly plaques with resulting pigmentary changes and scars; the plaques are frequently found on the face and scalp. DLE most commonly afflicts women in the third and fourth decades of life, although it may occur at any age. Individuals of African and Hispanic descent are at increased risk, and there may be a positive family history of lupus or connective tissue disease.

Discoid rash is one of the 11 diagnostic criteria for systemic lupus erythematosus (SLE), and 20% of patients with SLE will manifest discoid lesions. However, only 5%-10% of patients with DLE demonstrate systemic involvement or will go on to develop SLE. Risk factors for the development of SLE include widespread DLE, arthralgias ⁄ arthritis, nail changes, anemia, leukopenia, an elevated ESR, and a positive test for antinuclear antibodies (ANA).

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

The presence of erythema multiforme-like lesions in a patient with lupus, along with a speckled pattern of antinuclear antibody (ANA), positive anti-Ro/SSA or anti-La/SSB, and positive rheumatoid factor (RF) is known as Rowell syndrome. This syndrome has been described in patients with DLE, subacute cutaneous lupus erythematosus (SCLE), and SLE. Its existence as a distinct entity has been debated in the literature; some authors believe the association is coincidental. Prednisone with or without hydroxychloroquine, dapsone, or immunosuppressive drugs such as cyclosporin have been cited as therapy.

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