Rituximab shows promise in refractory lupus erythematosus panniculitis in children

Rituximab may be a promising option for treatment-refractory childhood-onset lupus erythematosus panniculitis, according to a retrospective, observational case series published in JAMA Dermatology.

“Childhood-onset lupus erythematosus panniculitis (LEP) is a rare and disfiguring skin disease affecting children. There are no standard approaches to treatment,” Colleen K. Correll, MD, MPH, assistant professor of pediatrics at the University of Minnesota Medical School and University of Minnesota Physicians pediatric rheumatologist, told Healio. “We have found that treatment with rituximab, an intravenous medication that targets B cells, successfully treated patients in our clinic who had failed other treatment medications.”

Researchers conducted a retrospective case series study of four patients with childhood-onset LEP from the University of Minnesota pediatric dermatology and rheumatology clinics between July 2014 and July 2018.

Patient one presented with recurrent, tender and disfiguring facial nodules with multiple T2-hyperintense, enhancing subcutaneous nodules of the face and scalp. This patient was treated with hydroxychloroquine, mycophenolate mofetil, azathioprine and then rituximab. Patient two had a 5-year history of tender subcutaneous nodules on his scalp, neck and upper back. He was treated with hydroxychloroquine, azathioprine and rituximab. Patient three had recurrent Kikuchi disease and severe atopy and presented with a 1-month history of facial lipoatrophy and erythematous, tender subcutaneous nodules on the face and neck. He was treated with prednisone and methotrexate initially before being treated with hydroxychloroquine and then, when there were no improvements, with rituximab. Patient four was the only girl in the cohort and had a 1-year history of tender, indurated nodules on the cheeks and temples. She was treated with hydroxychloroquine and mycophenolate mofetil but after no improvement was treated with rituximab.

Results showed that patient one had complete resolution of all skin nodules 6 weeks after treatment with rituximab, patient two had resolution of all nodules after treatment with rituximab 8 weeks after treatment, and patient three had resolution of facial nodules and improvement in lipoatrophy after follow-up at 1 month. Patient four had two minor reactions during the first administration of rituximab but tolerated the second administration well. Resolution of erythema, nodules and alopecia was found 6 months after treatment, but lipoatrophy remained.

Resolution of LEP after treatment with rituximab may imply an antibody-mediated role in the disease. The history of patient four’s disease may be different from the other patients as she was the only female patient, was younger and had concurrent alopecia areata, which may account for the failure for lipoatrophy improvement.

“A major problem with researching LEP and many other pediatric autoimmune diseases is that these diseases are very rare. It becomes a challenge to effectively study the causes and best treatments of these rare diseases when clinicians, researchers and institutions treat only a small number of these patients,” Correll said. “In order to establish the most effective studies, we often need larger populations of patients. To do this, we must collaborate with centers across the country and internationally.” – by Erin T. Welsh

Disclosures: Correll reports no relevant financial disclosures. Please see the study for all other authors’ relevant financial disclosures.