A dramatic case of non-episodic angioedema with eosinophilia

Abstract

Gleich Syndrome, also known as episodic angioedema with eosinophilia, was first described in 1984 by Gleich et al as a rare cause of hypereosinophilia. Angioedema with eosinophilia can be classified into 2 types: episodic or non-episodic. We present a dramatic case of non-episodic angioedema with eosinophilia in a young male. A 34 year old construction worker, presented to the emergency department in the summer of 2008 complaining of generalized fatigue, muscle aches and upper extremities swelling with no evidence of skin rash. He had been vacationing at his trailer in Southeastern Ontario, but denies any trauma, bug bites or new medications. Initial investigations showed a marginallly elevated WBC and eosinophils. He was discharged home on comfort measures only, but returned to hospital 2 days later complaining of shortness of breath, pleuritic chest pain and rapidly progressing generalized edema involving his face, neck, arms, upper chest and abdomen. His blood work showed markedly elevated WBC and eosinophilia peaking at 62.6x10(9) cells/L and 45.70x10(9) cells/L, respectively. A CT scan of the chest revealed bilateral pleural effusions, where as a cardiac MRI demonstrated a small pericardiac effusion. A bone marrow aspiration and biopsy confirmed marked hypereosinophilia with no leukemic features. A triceps muscle biopsy demonstrated marked eosinophilic infiltration. This patient responded well to oral steroids started at 1mg/kg with a slow tapering regimen and developed no permanent organ damage. Thorough work up failed to identify any cause for his condition, including a lack of FIP1L-PDGFRA gene. His WBC and eosinophil count returned to normal values in 3 months with no recurrence of angioedema for one year.