Angioedema episodic with eosinophilia T78.3

Author: Prof. Dr. med. Peter Altmeyer

Last updated on: 29.10.2020

Synonym(s)

Episodic angioedema associated with eosinophilia; Episodic angioedema with eosinophilia; Equal Syndrome; Equal syndromes; Gleich Syndrome

History
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Same, 1984

Definition
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Rare, chronically recurrent clinical picture with severe angioedema, urticaria and often very high peripheral (primary) eosinophilia (up to > 50,000/ul).

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Dermatology

Hypereosinophilia syndrome

The term HES covers a heterogeneous group of rare diseases (hypereosinophilia syndromes), whose characteris...

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Etiopathogenesis
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Unexplained; T-cell defects and functional disorders of the eosinophilic granulocytes have been demonstrated. Also occurred after taking ECE inhibitors.

Manifestation
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Different ages; young people can also be affected.

Clinical features
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Mostly dramatic, recurrent (monthly intervals; also more often), most severe swelling of the face, neck, extremities and trunk. These episodic angioedema can be combined with high fever attacks, arthralgias, weight loss. The episodes can also occur after infections.

Laboratory
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Marked eosinophilia (60-80%) in peripheral blood, elevated serum IgM.

Differential diagnosis
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Angioedema of other etiology, nonepisodic angioedema with eosinophilia, capillary leak syndrome.

Therapy
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The clinical picture responds well to higher and medium doses of glucocorticoids (e.g. prednisolone 1.0-1.5 mg/kg bw/day).

Occasionally, success with IVIG has also been described.

A case report is available on effects with "Suplatast tosilate", an anti-allergic Th2 cytokine inhibitor.

Note(s)
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A clinically similar clinical picture is seen with the " capillary leak syndrome" in the treatment of malignant tumours with interleukin-2. There are close relationships to the hypereosinophilia syndrome. In individual cases, episodic eosinophilic angioedema occurred in connection with the intake of ACE inhibitors (see eosinophilia below).

Literature
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Abouzahir A et al (2005) Equal syndromes. A case report and review of the literature. Rev Med Internal 26: 137-140
Banerji A et al (2006) Cytokine-associated angioedema syndromes including episodic angioedema with eosinophilia (Gleich's syndrome). Immunol Allergy Clin North Am 26: 769-81
Equals GJ, Schroeter AL, Marcoux JP, Sachs MI, O'Connell EJ, Kohler PF (1984) Episodic angioedema associated with eosinophilia. Trans Assoc Am Physicians. 97: 25-32
Hashizume H et al (2014) Treatment of non-episodic angioedema associated with eosinophilia with suplatast tosilate, an anti-allergic selective Th2 cytokine inhibitor. Eur J Dermatol 24:250-252
Motegi S et al (2013) Elevated serum levels of TARC/CCL17, eotaxin-3/CCL26 and vascular endothelial growth factor in a patient with non-episodic angioedema associated with eosinophilia and granulomatous cutaneous reaction. Eur J Dermatol 23:524-526
Panaszek B et al (2003) Blood and tissue eosinophilia in patient with angioedema caused by inhibitor of angiotensin converting enzymes (ACE). Pol Arch Med Wewn 110: 1339- 1343
Stockner I et al (2008) Non-episodic angioedema associated with eosinophilia following Mycoplasma pneumoniae infection. Clin Rheumatol 27:1573-1576