Researchers define characteristics of pediatric-onset discoid lupus erythematosus

Patients with pediatric discoid lupus erythematosus combined with systemic lupus erythematosus were most likely to be older and face single end-organ disease, according to data published in the Journal of American Academy of Dermatology.

“Discoid lupus erythematosus (DLE) can cause permanent scarring in cosmetically sensitive areas such as the face and scalp,” Nnenna Ezeh, MD, of the University of Wisconsin School of Medicine and Public Health at the time of the study, and colleagues wrote.

The researchers continued that pediatric DLE (pDLE) is rare with less than 3% of cases reported before age 10 years. Additionally, pediatric-onset systemic lupus erythematosus (SLE) differs from adult-onset SLE with more aggressive complications and a two-fold increased rate of mortality.

To better understand pDLE and SLE in children, Ezeh and colleagues conducted a multicenter retrospective study with the primary aim to characterize clinical outcomes to facilitate future development of a consensus treatment plan.

The cohort comprised 438 patients. Based on ACR classification, 63% had pDLE only and 37% had pDLE with SLE. Based on SLICC classification, 59% had pDLE only and 41% had pDLE with SLE.

The cohort was racially diverse with 35% Black, 22% Hispanic, 20% white and 9% Asian patients. However, the girl to boy ratio was 2.6 to 1.

Black (42%) and Asian patients (58%) were more likely than white (23%) and Hispanic patients (27%) to present with both pDLE and SLE (P < .001).

Patients with both pDLE and SLE were also more likely to be older at rash onset (12.9 vs. 8.9 years; P < .001), have a shorter delay from rash onset to diagnosis of DLE (2.3 vs. 6.6 months; P < .001), have seen a rheumatologist at baseline (P < .001) and have a family history of SLE (23% vs. 14%; P = .02) compared with the pDLE only group.

End-organ disease was common among patients with pediatric DLE and SLE, as 41% presented with single-end organ disease, such as arthritis, renal disease, seizures, psychosis, pleuritis or pericarditis. Another 23% presented with two or more organ systems involved.

“As expected, those with [pDLE with SLE] had more laboratory abnormalities reflecting immunologic, hematologic and renal disease, as well as end-organ dysfunction, as these are criteria for SLE,” Ezeh and colleagues wrote.

They added that children with an SLE diagnosis at baseline had a shorter time from rash onset to DLE diagnosis compared with the pediatric DLE only group, which could be due to systemic complaints or other factors such as socioeconomics or race.

“Because DLE is a scarring process and earlier treatment reduces permanent disease damage, this represents a critical area for improvement,” the researchers concluded. “Even a wait time of months may permanently scar a child in cosmetically sensitive areas at a time when body image and peer victimization may be significant concerns.”