PEDIATRICS Vol. 61 No. 1 January 1978, pp. 127-130
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Pneumatosis Intestinalis Associated With Fatal Childhood Dermatomyositis

Thomas J. Fischer M.D.1, Louis Cipel M.D.1, and E. Richard Stiehm M.D.1

1 Departments of Pediatrics and Radiology, University of California Los Angeles School of Medicine, Los Angeles, California

Gastrointestinal complications are frequent in childhood dermatomyositis.1 Pneumatosis intestinalis has been reported only twice and both children had a benign course.2,3 We now report a boy in whom the appearance of pneumatosis intestinalis preceded colonic perforation, peritonitis, and death.

CASE REPORT

A boy 8frac12 years of age presented with a one-month history of muscle pain, weakness, dysphagia, and periorbital swelling. A diagnosis of dermatomyositis was established by elevated muscle enzyme levels and an abnormal electromyogram and muscle biopsy specimen. Despite prednisone therapy (60 mg/day), respiratory impairment developed and intravenous methotrexate (1 mg/kg/wk) was added, resulting in clinical improvement. During the next nine months the patient was hospitalized twice for respiratory weakness and a third time for abdominal pain, for which he underwent a third time for abdominal pain, for which he underwent a laparotomy with an incidental appendectomy.




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