Article Text

PDF

Non-episodic angioedema associated with eosinophilia
  1. Kiyoshi Shikino1,
  2. Yuta Hirose1,
  3. Seitaro Nakagawa2,
  4. Masatomi Ikusaka1
  1. 1Department of General Medicine, Chiba University Hospital, Chiba, Chiba, Japan
  2. 2Department of Dermatology, Chiba University Hospital, Chiba City, Chiba, Japan
  1. Correspondence to Dr Kiyoshi Shikino, kshikino{at}gmail.com

Statistics from Altmetric.com

Description

A 26-year-old woman presented with a 1-week history of peripheral oedema, fever and polyarthralgia. Physical examination revealed symmetrical non-pitting oedema of her hands and legs (figure 1). Her medical history was unremarkable. Laboratory findings showed blood eosinophilia (3800/μL). The serum IgM level was within normal. Skin histopathology revealed slight interstitial oedema, dilated venous vessels with endothelial swelling and inflammatory cells extending into the subcutaneous tissue (figure 2). Her condition was diagnosed as non-episodic angioedema with eosinophilia (NEAE). Her symptoms quickly improved with prednisolone (15 mg/day). After tapering off of prednisolone, she has been recurrence-free for 2 years.

Figure 1

Symmetrical non-pitting oedema of her legs.

Figure 2

Histopathology of the skin showing slight interstitial oedema, dilated venous vessels with endothelial swelling and inflammatory cells extending into the subcutaneous tissue.

Angioedema with eosinophilia is classified as episodic angioedema with eosinophilia (EAE) or NEAE. EAE is characterised by recurrent episodes of angioedema, arthralgia and fever with a markedly high peripheral blood eosinophil count and high serum IgM level. NEAE is characterised by a single episode of persistent oedema of the extremities, peripheral eosinophilia, lack of an increased serum IgM level and a less severe clinical course than EAE.1 NEAE generally affects young Asian women.1 NEAE is also characterised by the effectiveness of low-dose prednisone or spontaneous remission.2 The differential diagnosis includes Drug Reaction with Eosinophila and Systemic Symptoms (DRESS) syndrome consisting of a severe rash, fever, lymphadenopathy, elevated liver enzymes and/or eosinophilia.3

Learning points

  • Angioedema with eosinophilia is classified as episodic angioedema with eosinophilia or non-episodic angioedema with eosinophilia (NEAE).

  • NEAE is characterised by a single episode of persistent oedema of the extremities, peripheral eosinophilia, lack of an increased serum IgM level.

  • NEAE is also characterised by the effectiveness of low-dose prednisone or spontaneous remission.

References

View Abstract

Footnotes

  • Twitter Follow Kiyoshi Shikino at @K

  • Contributors KS, YH, SN and MI were involved in managing the patient. KS wrote the first draft. SN made the pathology specimen. MI edited the manuscript.

  • Competing interests None declared.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.