# 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.

## Key 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.