Juvenile dermatomyositis skin disease more active than muscle involvement

Source/Disclosures Published by:

disclosures:
The authors report no relevant financial disclosures.

ADD TOPIC TO EMAIL ALERTS

Receive an email when new articles are posted

Enter your email address to receive an email when there are new articles on . ” data-action = “subscribe” > Subscribe

We were unable to process your request. Try again later. If this problem persists, please contact customerservice@slackinc.com.

Back to Healio

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 can lead to severe sequelae, including dystrophic calcifications, persistent skin inflammation, and organ involvement with functional impairment,” Andi Wang, M.A., of the Department 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 the duration of untreated disease, skin activity, and muscle activity in 184 children with juvenile dermatomyositis between 1971 and 2019.

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

Nine patients (4.9%) had calcifications at enrollment and an additional 22 patients developed calcifications. Of these 31 calcifications, 87% were mild and 77.4% had disappeared.

In 25% of the patients, calcification recurred despite therapy and in 22.6% of the patients this did not resolve.

The 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 over 48 years, this study provides a historical and comprehensive overview of the treatment of juvenile dermatomyositis,” the authors wrote. “The data confirm that juvenile dermatomyositis patients present with larger skin than muscle involvement and that skin features take longer to resolve.”

ADD TOPIC TO EMAIL ALERTS

Receive an email when new articles are posted

Enter your email address to receive an email when there are new articles on . ” data-action = “subscribe” > Subscribe

We were unable to process your request. Try again later. If this problem persists, please contact customerservice@slackinc.com.

Back to Healio

Comments are closed.