PRESENTATION OF HENOCH SCHONLEIN PURPURA IN AN ELDERLY MAN WITH END STAGE RENAL DISEASE

Abstract

SESSION TITLE: Tuesday Fellows Case Report Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Our patient had end stage renal disease secondary to hypertension and was diagnosed with Henoch Schonlein purpura following a skin biopsy. He presented with the classic tetrad of a purpuric rash, abdominal pain, joint involvement and ‘underlying’ renal disease. A biopsy of the rash on the skin revealed leukocytoclastic vasculitis (LCV with vascular deposition with IgA and complement C3 on immunofluorescence. The use of steroids was associated with a resolution of symptoms. His previous renal disease made diagnosis tricky but the skin biopsy made diagnosis certain. CASE PRESENTATION: 63-year-old male with a clinical history of end stage renal disease, hypertension and congestive heart failure was admitted for a two-week history of generalized weakness, bilateral knee pain and swelling a generalized multiple non-blanching, petechial and purpuric rash with bullae. He developed abdominal pain, vomiting and melena after admission. Laboratory results on admission included: hematocrit 29%; hemoglobin 9.1mg/dl, leukocyte count 13,700/mm³ (neutrophils 83%, lymphocytes 5%); ERS 73mm/hour; serum urea nitrogen 93mg/dl; creatinine 7.7 mg/dL and normal coagulation studies. C3 and C4 levels were reduced. An EGD/push enteroscopy showed vascular ectasias overlying the gastric antrum and ulceration. An interval EGD performed five days after showed improvement in antral vascular ectasia. Biopsies of the gastrointestinal tract showed small intestinal mucosa with intact villous architecture and mild chronic inactive gastritis. A skin biopsy of the rash on his right arm showed superficial dermatitis with mid perivascular neutrophil inflammation with focal fibrinoid necrosis and fragmented neutrophilic nuclei. PAS stain highlighted focal fibrinoid necrosis of the small vessel with fibrin extravasation, consistent with leukocytoclastic vasculitis (LCV); and vascular deposition with IgA and complement C3. He was started on oral Prednisone with resolution of gastrointestinal bleeding and clinical symptoms. His hospital course was complicated by sepsis and he died six weeks after admission. DISCUSSION: HSP is a small vessel vasculitis affecting the skin and other organs. It's more common in the pediatric population and the diagnosis in adults needs a high index of suspicion. An etiology was not identified in our patient but he did have three of four symptoms in the 1990 criteria for the classification of HSP by the American College of Rheumatology. HSP is known to cause renal failure with more severe renal manifestations in the elderly. Our patient already had underlying renal failure increasing his risk of morbidity and mortality making his diagnosis and treatment a challenge. CONCLUSIONS: HSP in adults with underlying renal disease makes diagnosis challenging. Having a high index of suspicion and obtaining a biopsy helps with establishment of a diagnosis and early treatment. Reference #1: Mills JA, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Henoch-Schönlein purpura. Arthritis Rheum 33: 1114-1121, 1990. Reference #2: Henoch-Schönlein Purpura in Adults: Outcome and Prognostic Factors Evangéline Pillebout, Eric Thervet, Gary Hill, Corinne Alberti, Philippe Vanhille, Dominique Nochy JASN May 2002, 13 (5) 1271-1278; DOI: 10.1097/01.ASN.0000013883.99976.22 DISCLOSURES: No relevant relationships by Yizhak Kupfer, source=Web Response No relevant relationships by Olaoluwatomi Lamikanra, source=Web Response No relevant relationships by Steve Obanor, source=Web Response No relevant relationships by Vignesh Ponnusamy, source=Web Response No relevant relationships by Chanaka Seneviratne, source=Web Response No relevant relationships by Karen Tuneev, source=Web Response No relevant relationships by Taek Sang Yoon, source=Web Response