Severe Juvenile Dermatomyositis With Peripheral Nervous System Involvement: A Case Report

Juvenile dermatomyositis is the most common inflammatory myopathy of childhood. While many cases achieve favorable outcomes, severe forms may develop persistent disease activity, complications, and long-term disability. A five-year-old girl was admitted after 20 days of Gottron's papules and heliotrope rash, with intermittent fever during the first 10 days. She then developed mild proximal-predominant muscle weakness in all four limbs, rapidly progressing to severe generalized weakness with signs of axonal neuropathy over the subsequent 15 days. Despite aggressive immunosuppression with IV methotrexate, high-dose methylprednisolone pulses, IV immunoglobulin, oral prednisolone, and azathioprine, she demonstrated inadequate clinical response over four years of follow-up. Severe juvenile dermatomyositis with peripheral nervous system involvement may follow a refractory course despite guideline-directed aggressive immunosuppression. This case highlights the therapeutic challenges of rapidly progressive disease with atypical neurological complications and underscores the need for early recognition and intensified treatment strategies in high-risk presentations.