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 Table of Contents  
CASE REPORT
Year : 2014  |  Volume : 6  |  Issue : 10  |  Page : 543-544

An unusual presentation of childhood vasculitis presenting in adulthood: A challenging diagnosis of Henoch-Schönlein Purpura


1 Mayo Clinic, Rochester, Minnesota, USA
2 Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA
3 Bassett Medical Center, Cooperstown, New York, USA

Date of Web Publication20-Oct-2014

Correspondence Address:
Wisit Cheungpasitporn
Mayo Clinic, Rochester, Minnesota-55905
USA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1947-2714.143289

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  Abstract 

Context: Henoch-Schφnlein purpura (HSP), a systemic IgA vascultitis, is uncommon in adults, with an incidence rate of 0.1 to 1.2 per million in adults over 20 years old. This vasculitic syndrome can present as an uncommon cause of intestinal obstruction in older patients. We report a case of an older woman with HSP presenting with small bowel obstruction and vasculitic rash. Case Report: We report a 67-year-old woman who presented with small bowel obstruction and skin rash. Skin biopsy revealed leukocytoclastic vasculitis with +IgA granular deposition within the walls of superficial dermal vessels. Kidney biopsy confirmed the diagnosis of HSP with mild mesangial proliferative IgA nephropathy. Her abdominal pain and small bowel obstruction were improved with conservative treatment. She continued to do well with normal kidney function at a 3-month follow-up visit. Conclusion: HSP, a systemic IgA vasculitis, is a predominantly pediatric vasculitis and is uncommon in adults. In adults, the disease process is identical to that in children. However, gastrointestinal manifestation is less common in older patients, and bowel perforation and obstruction are rare. Intestinal obstruction with skin rash and renal involvement should raise suspicions of HSP.

Keywords: Henoch-Schönlein purpura, Intestinal obstruction, Vasculitis


How to cite this article:
Thongprayoon C, Cheungpasitporn W, Thamcharoen N, Bruminhent J. An unusual presentation of childhood vasculitis presenting in adulthood: A challenging diagnosis of Henoch-Schönlein Purpura. North Am J Med Sci 2014;6:543-4

How to cite this URL:
Thongprayoon C, Cheungpasitporn W, Thamcharoen N, Bruminhent J. An unusual presentation of childhood vasculitis presenting in adulthood: A challenging diagnosis of Henoch-Schönlein Purpura. North Am J Med Sci [serial online] 2014 [cited 2019 Nov 11];6:543-4. Available from: http://www.najms.org/text.asp?2014/6/10/543/143289


  Introduction Top


IgA Nephropathy (IgAN) is the most common primary glomerulonephritis (GN) in the world. [1],[2],[3] There is a peak incidence of IgAN in the second and third decades of life. Henoch-Schφnlein purpura (HSP) is uncommon in adults, with an incidence rate of 0.1-1.2 per million in adults over 20 years old. [4] This vasculitic syndrome can present as an uncommon cause of intestinal obstruction in older patients. We report a case of an older woman with HSP. She presented with small bowel obstruction and purpuric rash over of the lower extremities.


  Case Presentation Top


A 67-year-old woman presented to the hospital with a 7-day history of severe, diffuse abdominal pain associated with nausea and vomiting. She simultaneously developed pain involving both her elbows with difficulty bending her arms and a blotchy red purpuric rash over of the lower extremities knee down [Figure 1].
Figure 1: A blotchy red purpuric rash over of the lower extremities knee down

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Physical examination revealed an abdominal distention with increased bowel sound. There was diffuse tenderness without guarding or rebound tenderness. Initial laboratory investigations revealed no leukocytosis with normal serum creatinine of 0.7 mg/dL. Urinalysis demonstrated microscopic hematuria and mild proteinuria.

Computed tomography (CT) of abdomen was performed [Figure 2] and demonstrated a segment of bowel edema with partial small bowel obstruction. Skin biopsy revealed leukocytoclastic vasculitis involving the small vessels. A direct immunofluorescent technique showed +IgA granular deposition within the walls of many superficial dermal vessels. She underwent kidney biopsy for evaluation for vasculitis, which confirmed the diagnosis of HSP with mild mesangial proliferative IgAN. She had a bowel rest and a nasogastric tube was placed for her partial small bowel obstruction. Her abdominal pain and small bowel obstruction were improved with conservative treatment. Follow-up CT abdomen showed completely resolved bowel edema. The patient chose closed follow up without immunosuppression. She continued to do well at a 3-month follow-up visit.
Figure 2: Computed tomography (CT) of the abdomen demonstrated a segment of bowel edema with partial small bowel obstruction

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  Discussion Top


IgA Nephropathy (IgAN) is the most common primary glomerulonephritis (GN) in the world. [3] Recently, a meta-analysis of 6,543 patients with IgAN demonstrated an increase in incidence of ESRD in the older group. [5]

HSP, a systemic IgA vasculitis, is a predominantly pediatric vasculitis. Ninety percent of cases occur in the pediatric age group between the ages of 3 and 15 years. HSP occurs uncommonly in adults with an incidence rate of 0.1-1.2 per million in adults over 20 years old. [4] In adults, the disease process is identical to that in children. However, gastrointestinal manifestation is less common in older patients, and bowel perforation and obstruction are rare. [6],[7] Intestinal obstruction with skin rash and renal involvement should raise the suspicions of HSP.

 
  References Top

1.Barratt J, Feehally J. IgA nephropathy. J Am Soc Nephrol 2005;16:2088-97.  Back to cited text no. 1
    
2.D'Amico G. The commonest glomerulonephritis in the world: IgA nephropathy. Q J Med 1987;64:709-27.  Back to cited text no. 2
    
3.Wyatt RJ, Julian BA. IgA nephropathy. N Engl J Med 2013;368:2402-14.  Back to cited text no. 3
    
4.Watts RA, Carruthers DM, Scott DG. Epidemiology of systemic vasculitis: Changing incidence or definition? Semin Arthritis Rheum 1995;25:28-34.  Back to cited text no. 4
    
5.Duan ZY, Cai GY, Chen YZ, Liang S, Liu SW, Wu J, et al. Aging promotes progression of IgA nephropathy: A systematic review and meta-analysis. Am J Nephrol 2013;38:241-52.  Back to cited text no. 5
    
6.Cheungpasitporn W, Jirajariyavej T, Howarth CB, Rosen RM. Henoch-Schonlein purpura in an older man presenting as rectal bleeding and IgA mesangioproliferative glomerulonephritis: A case report. J Med Case Rep 2011;5:364.  Back to cited text no. 6
    
7.Chung DJ, Park YS, Huh KC, Kim JH. Radiologic findings of gastrointestinal complications in an adult patient with Henoch-Schonlein purpura. AJR Am J Roentgenol 2006;187:W396-8.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2]



 

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