Gaining Weight And Loosing Appetite! ; A Case Of Recurrent Diffuse Angioedema, Weight Gain and Peripheral Eosinophilia Without Visceral Organ Damage

Abstract

Gleich's syndrome is a variant of Hypereosinophilic syndrome (HES) presenting with episodic angioedema, urticaria, fever, anorexia and significant weight gain but lacking visceral involvement seen in classic HES. History, clinical exam, appropriate laboratory tests. Patient presented with five years of episodic generalized facial, extremities and trunk swelling accompanied by weight gain, fever, urticaria and anorexia. Each episode developed over a few days and lasted for 2-3 months, initially occurring biannually. Recent episodes have occured more frequently.Initial workup showed 16.31K/mm3 WBC with 8,644 normal appearing eosinophils. Strongyloides titer was negative. FIP1L1/PRGFR fusion by FISH was negative. C3, C4, C1 Inhibitor functional assay, metabolic profile and TSH were all normal. IgE was 804 IU/mL but other immunoglobulins were normal. ANA and ANCA were negative. CT showed esophageal, supraglottic and glottic edema. Esophagogastroduodenoscopy with biopsies showed no pathology. In absence of findings for other cause of eosinophilia and no organ involvement, a diagnosis of Gleich's syndrome was made. Patient responded to prednisone with hydorxyurea added as a steroid sparing agent. Gleich's syndrome should be considered in patients with episodic angioedema. Hypereosinophilia from other causes is associated with angioedema and should be ruled out. Lack of visceral involvement is the key to diagnosing this variant of HES.