We describe a 42-year-old man with dermatomyositis and interstitial lung di
sease who presented with anterior neck pain and dyspnoea. Chest radiographs
showed subcutaneous emphysema, pneumomediastinum and diffuse reticulonodul
ar infiltration in both lungs. After the administration of high doses of pr
ednisolone, an improvement of pulmonary function and respiratory symptoms w
as observed but the pneumomediastinum persists 12 months after diagnosis, a
nd without any complication. We review the cases that have been reported th
us far of pneumomediastinum associated with dermatomyositis and discuss the
possible mechanisms involved. We conclude that pneumomediastinum is not an
uncommon complication of dermatomyositis and that its aetiopathogenesis re
mains very unclear.