Angioedema episodic with eosinophilia T78.3

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

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.

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.

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