A 6-year-old girl has 2 months of progressive difficulty climbing stairs and combing her hair. Examination shows violaceous discoloration of the upper eyelids with periorbital edema, scaly violaceous papules over the MCP knuckles, and dilated nailfold capillary loops. Serum CK is elevated. What is the most likely diagnosis?

• A Polymyalgia rheumatica
• B Duchenne muscular dystrophy
• C Systemic lupus erythematosus
• D Juvenile dermatomyositis ✓

Explanation

Symmetric proximal weakness with a heliotrope rash of the eyelids, Gottron papules over the knuckles, nailfold capillary changes, and raised muscle enzymes define juvenile dermatomyositis. Duchenne dystrophy causes proximal weakness but never produces these pathognomonic skin signs, and it affects boys through dystrophin mutation. SLE can have rashes but not Gottron papules or this pattern of myositis.

Reference: Nelson Textbook of Pediatrics, 21st ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.