Juvenile dermatomyositis skin disease more active than muscle involvement

Skin disease is more active than muscle disease in juvenile dermatomyositis and should be treated early and aggressively to limit its severity, according to a study.

“Juvenile dermatomyositis may lead to severe sequelae, including dystrophic calcifications, persistent cutaneous inflammation and organ involvement with functional limitations,” Andi Wang, MA, of the division of pediatric rheumatology, department of pediatrics, at Northwestern University Feinberg School of Medicine, and colleagues wrote.

A prospective cohort study used data from the Juvenile Myositis Registry to evaluate duration of untreated disease, skin activity and muscle activity in 184 children with juvenile dermatomyositis between 1971 and 2019.

Skin involvement at entry was minimal in 0.6% of patients, mild in 45.2%, moderate in 45.2% and severe in 9%. Vascular involvement, including eyelid margin vessel dilation, eyelid erythema, periungual capillary telangiectasia or erythema, or palatal vessel dilation, was recorded in more than 99% of patients.

Nine patients (4.9%) had calcifications at enrollment with 22 additional patients developing calcifications. Of these 31 calcifications, 87% were mild and 77.4% resolved.

Calcification recurred despite therapy in 25% of patients and failed to resolve in 22.6% of patients.

Treatments included oral prednisone (89%), intravenous methylprednisolone (70%), methotrexate (68%), hydroxychloroquine (35%), mycophenolate mofetil (29%), cyclosporine (15%) and intravenous immunoglobulin (1%).

“Given the collection of data for 48 years, this study provides a historical and comprehensive look at the management of juvenile dermatomyositis,” the authors wrote. “The data confirm that juvenile dermatomyositis patients present with greater skin than muscle involvement and that skin features require more time for resolution.”