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Massive gastrointestinal haemorrhage in isolated intestinal Henoch-Schonlein purpura with response to intravenous immunoglobulin infusion

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Abstract

There is increasing recognition that Henoch-Schonlein purpura may present in an atypical form in which gastrointestinal symptoms may predominate, and classic cutaneous changes may be delayed or absent. This may lead to significant diagnostic delay. We report the case of a 9-year-old girl who presented acutely with life-threatening gastrointestinal haemorrhage from multiple intestinal sites, with no skin rash and only mild evidence of renal involvement. Henoch-Schonlein purpura was confirmed by finding IgA deposition on vessels within gastric and duodenal mucosa, while immunohistochemistry also identified dense focal T cell infiltration in gastric mucosa and within duodenal epithelium. After initial stabilisation, the patient became shocked due to further gastrointestinal haemorrhage. Isotope bleeding scan identified multiple bleeding sites. Her endoscopically confirmed gastritis was sufficiently severe to preclude corticosteroids, and she was thus treated with intravenous immunoglobulin. This therapy induced prompt and sustained resolution of symptoms, and she has remained well since. Our patient’s response concords with previous reports in corticosteroid-resistant cases to suggest that severe intestinal Henoch-Schonlein purpura may respond preferentially to intravenous immunoglobulin (IVIG) therapy. In severe cases where there is significant gastritis, IVIG provides an effective alternative to corticosteroids that may be employed as first-line therapy.

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Abbreviations

HSP:

Henoch-Schonlein purpura

HSPG:

heparan sulphate proteoglycans

GAGs:

glycosaminoglycans

IL-1β:

interleukin-1β

TGF-β:

transforming growth factor-β

CRP:

c-reactive protein

IEL:

intraepithelial lymphocyte

IVIG:

intravenous immunoglobulin

URTI:

upper respiratory tract infection

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Authors and Affiliations

  1. Centre for Paediatric Gastroenterology, Royal Free and University College Medical School, London, UK

    Andrew A. O. Fagbemi, Franco Torrente & Robert B. Heuschkel

  2. Department of Nuclear Medicine, Royal Free Hampstead NHS Trust, London, UK

    Andrew J. W. Hilson

  3. Sheffield Children’s Hospital, Sheffield, UK

    Michael A. Thomson

  4. Clinical Sciences Research Institute, Warwick Medical School, Clifford Bridge Road, Coventry, CV2 2DX, UK

    Simon H. Murch

Authors
  1. Andrew A. O. Fagbemi
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  2. Franco Torrente
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  3. Andrew J. W. Hilson
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  4. Michael A. Thomson
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  5. Robert B. Heuschkel
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  6. Simon H. Murch
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Corresponding author

Correspondence to Simon H. Murch.

Additional information

Drs Fagbemi and Torrente contributed equally to this report, and should be considered as joint first authors.

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Fagbemi, A.A.O., Torrente, F., Hilson, A.J.W. et al. Massive gastrointestinal haemorrhage in isolated intestinal Henoch-Schonlein purpura with response to intravenous immunoglobulin infusion. Eur J Pediatr 166, 915–919 (2007). https://doi.org/10.1007/s00431-006-0337-3

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  • DOI: https://doi.org/10.1007/s00431-006-0337-3

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