Una dermatomiosite complessa

The paper presents the case of an 8-year-old girl that was admitted to the hospital with pruritic, erythematous and scaling skin lesions on her limbs, chest, back and fingers (Gottron’s papules), along with a malar (‘butterfly’) rash. She also had muscle pain, weakness and walking difficulty. Laboratory tests showed markedly elevated muscle enzymes (CPK, AST/ALT, CK-MB and troponin T), and autoimmune screening was positive for ANA (1:2560, granular pattern). Imaging (whole-body MRI) revealed inflammation of her leg muscles. She was diagnosed with juvenile dermatomyositis and treated initially with intravenous immunoglobulin (IVIg) and corticosteroids, followed by hydroxychloroquine (useful especially in resistant cutaneous and articular manifestations) and methotrexate due to insufficient response. Her condition gradually improved, but a later disease flare required adding mycophenolate mofetil and restarting oral steroids. She continues regular follow-up and monthly IVIg therapy, with overall clinical improvement.