Pediatric Discoid Lupus Erythematosus

Jeffrey P. Callen, MD reviewing Arkin LM et al. J Am Acad Dermatol 2015 Jan 30.

Clinical features, relationship to systemic disease, and risk for progression were assessed in a relatively large cohort of children.

Discoid lupus erythematosus (DLE) has been studied in adults, but its relative rarity in children has produced few studies documenting its clinical features and the risk for progression to systemic disease (SLE). In adults, the risk for serious end-organ damage (renal or central nervous system disease) is lower in patients with DLE than in unselected patients with SLE.

These authors retrospectively reviewed the cases of 40 patients younger than age 16 years diagnosed with DLE at a tertiary referral center between 1995 and 2012. Of these patients, six had concurrent diagnoses of DLE and SLE. Of the remaining 34 patients, 9 developed SLE as defined by American College of Rheumatology criteria during the mean 5 years of follow-up, and 15 developed some serologic or laboratory features of SLE but did not fulfill the criteria. Ten patients had disease limited to skin only. The greatest risk for developing SLE was in the first year following DLE diagnosis. In most patients who did meet the criteria, the pertinent criteria were mucocutaneous disease combined with serologic abnormalities or cytopenias. The authors point out several limitations of their observations, including that patients seen at a tertiary referral center might be more likely to have systemic disease and the lack of long-term follow-up (many adolescents transition to adult care).

Citation(s):

Arkin LM et al. The natural history of pediatric-onset discoid lupus erythematosus. J Am Acad Dermatol 2015 Jan 30; [e-pub ahead of print].