Rheumatology
en POLSKI
eISSN: 2084-9834
ISSN: 0034-6233
Reumatologia/Rheumatology
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1/2012
vol. 50
 
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abstract:
Case report

Severe course of Henoch-Schönlein purpura with gastrointestinal tract involvement

Rafał Małecki
,
Karolina Stawarska
,
Marek Frankowski
,
Alicja Markowska-Woyciechowska
,
Rajmund Adamiec

Reumatologia 2012; 50, 1: 61–65
Online publish date: 2012/03/02
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Henoch-Schönlein purpura (HSP) is a systemic small vessel vasculitis caused by IgA deposits. In the course of the disease, involvement of the skin, gastrointestinal tract, kidneys and joints is observed (classification criteria – Table I). HSP typically occurs in children (with a peak incidence in 4–5-year-olds) and is characterized by a mild, self-limited course, with a complete recovery within a few weeks or months. It is believed that rarely observed onset of HSP in the later age is connected with higher percentage of severe complications, often requiring aggressive treatment. We present a case of a 20-year-old man with HSP, who had a typical clinical manifestation including involvement of kidneys, skin (Fig. 1) and joints, and developed severe intestinal complications of HSP. Regardless of active treatment, persistent intestinal haemorrhage resulted in disseminated intravascular coagulation (DIC) and multiple organ insufficiency syndrome. Administered treatment including resection of a necrotic fragment of the small intestine (Fig. 2–4) did not cause clinical improvement. Despite treatment in the Intensive Care Unit, the patient died.
keywords:

Henoch-Schönlein purpura, gastrointestinal haemorrhage, disseminated intravascular coagulation




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