PP 13. Atypical Henoch-Schoenlein Purpura? Consider Polyarteritis Nodosa!


S. Braungart, A Campbell, S. Besarovic

19:22 - 19:28h at Margrit Room

Categories: Gastrointestinal Surgery, Poster Session

Session: Poster (P1) - Gastrointestinal Surgery


Background
Henoch-Schoenlein Purpura (HSP) is the most common systemic vasculitis in children. Abdominal pain is common in children with HSP. It often leads to surgical review to rule out surgical emergencies such as intussusception or bowel infarction. Polyarteritis Nodosa (PAN) - a systemic necrotizing small-vessel-vasculitis - is rare in children. Similar to HSP it can manifest itself with a variety of symptoms, including a wide spectrum of gastrointestinal complications.

Material and Methods
We report the case of a boy who initially presented with features suggestive of HSP but dramatic systemic organ involvement. He was finally diagnosed with PAN.

Result
Case report
A 12-year-old boy presented with features suggestive of HSP: generalized rash, colicky abdominal pain, fresh rectal bleeding. He developed dramatic systemic organ involvement shortly after admission including hypertension, proteinuria, pleural effusions. Serial ultrasounds were suggestive of bowel ischaemia. He underwent repeat laparotomy and resection of ischaemic bowel, with slow improvement following the second laparotomy. The severity of systemic involvement with persistent hypertension and proteinuria made the diagnosis of HSP questionable. Immunohistochemistry of skin biopsies was negative for HSP. Polyarteritis Nodosa was finally diagnosed from histopathology of the bowel specimen.

Conclusion
With this report we want to raise awareness of the possibility of early PAN mimicking HSP. In cases of severe systemic organ involvement this rare, but potentially fatal systemic vasculitis must be considered as a differential diagnosis which requires a high level of suspicion. Close collaboration between Surgeons and Paediatricians is paramount to instigate appropriate investigations/treatment in a timely fashion.