Ascites in a patient with episodic angio-oedema and eosinophilia: thinking outside the box

BMJ Case Rep. 2017 Apr 21:2017:bcr2017219467. doi: 10.1136/bcr-2017-219467.

Abstract

Episodic angio-oedema with eosinophilia (EAE) or Gleich's syndrome is a rare condition characterised by recurrent episodes of oedema and eosinophilia, accompanied by urticaria, fever and weight gain. The presence of ascites has not been reported so far. We report a 21-year-old Caucasian woman who presented with marked ocular oedema and ascites. Laboratory evaluation revealed marked eosinophilia. During the last 3 months, three episodes of facial and neck oedema were reported, which resolved spontaneously over a period of 3-5 days. The diagnosis of EAE was established after exclusion of secondary causes (infections, allergic reactions, collagen diseases, neoplasms) and clonal disorders associated with marked eosinophilia. Low-dose steroids resulted in eosinophil decrease and complete resolution of symptoms, including ascites. This case highlights that ascites can be a very rare manifestation of EAE particularly if other more frequent causes of ascites have been excluded and the clinical and laboratory findings are supportive of EAE.

Keywords: Immunology; Medical management.

Publication types

  • Case Reports

MeSH terms

  • Angioedema / diagnosis*
  • Angioedema / drug therapy
  • Ascites / etiology*
  • Eosinophilia / diagnosis*
  • Eosinophilia / drug therapy
  • Female
  • Humans
  • Steroids / therapeutic use
  • Treatment Outcome
  • Young Adult

Substances

  • Steroids