Introduction: Juvenile Dermatomyostis, is an inflammatory myopathy distinguished by proximal muscle weakness with cutaneous manifestations.
Gastrointestinal ulceration is a rare manifestation of JDM but can be life-threatening.
We report a case of girl with JDM, presented with sever intestinal perforation with concomitant CNS involvement.
Case: A 10 year-old-girl, Two months prior to her admission was diagnosed with JDM, treated with immunosuppressant therapy.
Three days prior to her admission, started to have right lower quadrant pain, vomiting and Chest pain aggravated by inspiration.
On physical examination, lipodystrophy, muscle wasting, muscle weakness, heliotrope rash, Gottron`s sign and diffuse abdominal tenderness.
On CT scan, free air and fluid were seen retroperitoneally in the right abdomen . therefore underwent appendectomy.
Continued to have abdominal pain, on revision of the CT, retroperitoneal perforation of the duodenum was identified.
later, the patient experienced tonic clonic seizure. CSF analysis and CT angiography were normal. Due to suspicion of vasculitic involvement of the brain, immunosuppressant therapy were added.
Discussion: In JDM, abdominal pain should be evaluated carefully, with ulceration and perforation included in the differential diagnosis .
Acute gastrointestinal vasculitis and chronic abdominal endarteropathy have been described in patients with JDM .
CNS involvement is rarely reported in JDM, which frequently occurs in other childhood autoimmune disease with systemic vasculitis.
Although biopsy of the brain is the gold standard of diagnosis, non-invasive studies in tandem with lumbar puncture have been demonstrated to have a high sensitivity.
