Juvenile dermatomyositis (JDMS) is an inflammatory illness of unknown etiology that primarily affects skin, muscle, and blood vessels. Children   usually present clinically with a characteristic rash and evidence of muscle weakness. Definitive diagnosis according to established criteria requires the presence of rash and evidence of myositis (proximal weakness, elevated muscle-related enzymes, characteristic electromyogram [EMG] or biopsy). Patients with the rash of dermatomyositis without muscle involvement (called amyopathic dermatomyositis or dermatomyositis sine myositis) have been described,3-7  but few are children. There is not clear agreement on whether the absence of clinical weakness alone is sufficient to place patients in this diagnostic category, or whether other investigations are needed to prove the absence of myositis. We describe five children who presented with the characteristic rash of JDMS, but without initial symptoms of muscle disease, all of whom are known to have developed myositis.

The five patients were...

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