K. Gerlach, P. Göbel  

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5 Years  

Male  

Generalized diseases  

unknown  

A 5 year old boy presented in the emergency room because of persistent abdominal pain for 5 days with one episode of vomiting.
Initially, the clinical picture was that of a nonspecific mesenteric lymphadenitis. Labs demonstrated leukocytosis of 24.00 Gpt/l with deviation to the left. Platelet count was 434.00 Gpt/l and CRP was 34 mg/l. ESR was increased (10 mm/h) with mild hypoproteinemia. Urinalysis was normal except for a few epithelial cells and bacteria.
Ultrasound demonstrated an inflammatory thickened small bowel wall with a cockade like structure in the right mid abdomen. This bowel wall thickening was also visible in the left mid and lower abdomen with increasing amount of free intraabdominal fluid during the hospital stay.
A CT of the abdomen was performed with the indication of an inflammatory pseudotumor, DD Burkitt lymphoma and a subileus. The boy underwent a diagnostic laparotomy and resection of a purple and thickened jejunal loop with end to end anastomosis.  

Histologically a hemorrhagic small bowel infarction with purulent lymphangitis was found. A second Pathology report described the picture of a submucosal leukocytoclastic vasculitis of the small vessels.

Henoch-Schoenlein Purpura is a generalized immune complex vasculitis of the small vessels of unknown etiology.After simple infections, an increased production of IgA, which if possibly due to hypersensitivity to bacterial antigenes (e.g. Streptococci), leads to deposition of immune complexes and to aseptic inflammation in different organ systems. These include skin (affected up to 100%) with palpable purpura, gastrointestinal tract (30-75%), joints, kidneys (6-60%), testis and CNS.  

<- view Ultrasound 1

Ultrasound 1: Wall thickened, hypervascular small bowel loop in the axial plane.





<- view CT 1

CT 1: Significantly wall thickened segment of jejunum in the left lower and mid abdomen with diffuse surrounding inflammatory reaction, lymphadenopathy and ascites.





<- view CT 2

CT 2: Significantly wall thickened segment of jejunum in the left lower and mid abdomen with diffuse surrounding inflammatory reaction, lymphadenopathy and ascites.





<- view OP-Situs 1

OP-Situs 1: Inflammatory changes and purple coloration of a small bowel loop, presenting as pseudotumor.





<- view Histology 1

Histology 1: Hemorrhagic necrotising enteritis with leukocytoclastic vasculitis.

 

Surgery / Histo  

Invagination, mesenteric lymphadenitis, inflammatory pseudotumor, Burkitt lymphoma  

Henoch-Schoenlein Purpura presents preferably in the cold seasons in the the jung childhood (5-7 years of age), with a slight predominance for boys. The diagnosis is made based on clinical symptoms. Henoch-Schoenlein Purpura is diagnosed if two of four of the following criteria (ACR) are present: palpable purpura, age of manifestation < 20 years, angina abdominalis (abdominal pain and blood in stool), presence of granulocytes in the vessel wall. The treatment is targeted to the symptoms, the prognosis is good.

After initial improvement, the boy developed recurrent swelling in feet and lower legs, testicular pain, massive thrombocytosis as well as position dependent petechia. During the further course a micro hematuria has developed. After administration of Prednisolone a complete cure could be accomplished.  

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1. Medline:
Chan JC,Li PK; Lai FM, Lai KN.
Fatal adult Henoch-Schönlein purpura due to small intestinal infarction.
J Intern Med 232(1992)181-4

2. Medline:
Prenzel F, Pfäffle R, Thiele F, Schuster.
Decreased factor XIII activity during severe Henoch-Schoenlein purpura - Does it play a role?
V. Klin Padiatr 2006;218:174-76

3. Medline:
Lawes D, Wood J.
Acute abdomen in Henoch-Schönlein purpura.
J R Soc Med. 2002 Oct;95(10):505- 6

4. Medline:
C.Rieger.
Vaskulitiden, In: Pädiatrie Grundlagen und Praxis. Hrsg. Lentze, Schaub, Schulte, Spanger, S.662ff. 2. Auflage 2003, Springer Verlag, Berlin, Heidelberg, New York,
 

Henoch-Schoenlein Purpura, small bowel infarct, thrombocytosis, petechia, child, childhood, pediatric radiology  

K Gerlach, P Göbel. Henoch-Schoenlein Purpura. PedRad [serial online] vol 8, no. 3.
URL: www.PedRad.info/?search=20080327175230