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

TL;DR

A five-year-old girl with severe juvenile dermatomyositis and peripheral nerve involvement showed poor response to aggressive treatment, highlighting the challenges of this rare condition.

Contribution

This case report documents a rare and refractory form of juvenile dermatomyositis with peripheral nervous system involvement.

Findings

The patient developed severe generalized muscle weakness and axonal neuropathy within 15 days.

Aggressive immunosuppressive therapy failed to produce a significant clinical response over four years.

The case emphasizes the need for early recognition and intensified treatment in high-risk presentations.

Abstract

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.

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