Abstract

Henoch-Schönlein purpura (HSP) is a small vessel systemic vasculitis, predominantly affecting children, characterized by a tetrad of manifestations, specifically palpable purpura, arthralgia, abdominal pain, and renal disease. HSP in the adult population is rare, and no case has been described of HSP in liver cirrhosis with spontaneous bacterial peritonitis (SBP). We present the case of a 58-year-old male with liver cirrhosis, who was subsequently diagnosed with SBP and later HSP. In this patient, the diagnosis of HSP was demonstrated clinically by his palpable purpura, diarrhea, hematuria, and abdominal pain and confirmed pathologically by his renal and skin biopsies demonstrating leukocytoclastic vasculitis and IgA complexes. We believe that this is an example of altered IgA processing in cirrhosis leading to the development of IgA immune complexes and ultimately HSP. The patient additionally had SBP, which may have increased his risk for developing HSP given antigen processing by mucosa-associated lymphoid tissues leading to immune complex deposition, which may not have been effectively cleared in the context of his liver disease. The patient unfortunately died of gastrointestinal hemorrhage, which is unclear to be due to his underlying cirrhosis or a gastrointestinal manifestation of HSP itself.

Highlights

  • Henoch-Schonlein purpura (HSP) is a small vessel systemic vasculitis, predominantly affecting children, with ninety percent of cases occurring in the pediatric population

  • We present the case of a 58-year-old male with liver cirrhosis and spontaneous bacterial peritonitis (SBP), confounded by the diagnosis of HSP

  • His renal failure may have been worsened by SBP and underlying cirrhosis, in addition to his IgA nephropathy. His ultimate hematemesis may have been due to variceal hemorrhage in the context of his liver cirrhosis, though gastrointestinal hemorrhage is known to be a rare manifestation of HSP. This is a unique case of significant HSP with both renal and skin manifestations along with SBP in a patient with underlying liver cirrhosis

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Summary

Introduction

Henoch-Schonlein purpura (HSP) is a small vessel systemic vasculitis, predominantly affecting children, with ninety percent of cases occurring in the pediatric population. HSP is characterized by a tetrad of manifestations, palpable purpura, arthralgia, abdominal pain, and renal disease, with gastrointestinal and renal involvement more prevalent in older adults [1, 2]. Gastrointestinal symptoms of HSP can range from mild symptoms of nausea, vomiting, or abdominal pain to gastrointestinal hemorrhage, bowel ischemia, and bowel perforation [3]. HSP in the adult population is rare, and no case has been described of HSP in liver cirrhosis with spontaneous bacterial peritonitis (SBP). We present the case of a 58-year-old male with liver cirrhosis and SBP, confounded by the diagnosis of HSP

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