Severe gastrointestinal manifestations of Henoch-Schönlein Purpura (HSP). Case report

Introduction: HSP is a systemic small vessel vasculitis characterized by vascular and/or mesangial IgA deposits, primarily affecting the vasculature of the skin, joints, kidneys and GI tract. Gastrointestinal findings of various severities occur in 50–85% of HSP patients. We report on a 70 year old woman who developed ileocoecal invagination and upper gastrointestinal haemorrhage as manifestations of HSP.

Case history: The patient presented with two days' history of purpuric rash localized on the lower extremities. Based on the result of the skin biopsy, which showed leukocytoclastic vasculitis, IgA and C3 deposits, HSP was suspected.

On the second night after admission colicky abdominal pain, vomiting and diarrhea developed. Radiological investigations proved ileocoecal invagination and as symptoms deteriorated coecum resection was performed. On the third postoperative day the patient became oligo-anuric and since the renal failure was also contributed to HSP, parenteral steroid therapy was instituted (500mg/die methyl-prednisolone for 3 days, then tapering the dose to 48mg/die). One month after initial admission the patient experienced hematochezia. Urgent upper endoscopy revealed petechiae, hemorrhagic erosions and mucosal oedema, primarily in the second part of the duodenum, but also present in the antrum and corpus of the stomach. Since these lesions were also considered as presentations of HSP, which has later been confirmed by histological examination of duodenal biopsy samples, another bolus of parenteral steroids was administered. Upper endoscopy carried out 3 days later showed remarkable improvement of the gastric and intestinal lesions.

Discussion: In HSP patients gastrointestinal symptoms are common manifestations of the underlying disease, thus they should not be overlooked. Administration of steroids is an effective therapeutical option not only in the management of severe renal symptoms, but also in the treatment of gastrointestinal manifestations.