Episodic Angioedema with Hypereosinophilia (Gleich's Syndrome): A Case Report and Extensive Review of the Literature.

Ilaria Mormile,Giuseppe Spadaro,Stefania Loffredo,Francesca Wanda Rossi,Maria Bova,Amato De Paulis,Mauro Mormile,Angelica Petraroli,Andrea Del Mastro

doi:10.3390/jcm10071442

Abstract

Episodic angioedema with eosinophilia (EAE) (Gleich’s syndrome) is a rare disease characterized by hypereosinophilia (up to 95 × 109 cells/L), recurrent episodes of angioedema, urticaria, weight gain, and fever, that occur at periodical intervals (usually every 3–4 weeks). The exact etiology of EAE is still unclear, but both eosinophils and abnormalities of cytokines homeostasis seem to play a pivotal role in the pathogenesis of the disease. In particular, the cyclic elevation of serum interleukin-5 before the increase in eosinophil count has been reported. Herein, we performed a broad literature review and report the case of a thirty-two-year-old woman with a two-year history of cyclic angioedema attacks, urticaria, periodic weight gain, and severe hypereosinophilia, diagnosed with EAE and treated with oral corticosteroids. Describing the most relevant clinical features of EAE reported so far in the literature, we aim to provide physicians with some useful tools to help them deal with this disease. In addition, we aim to raise awareness about this rare condition in which approved diagnostic classification criteria are currently missing.

Highlights

Introduction with regard to jurisdictional claims in Episodic angioedema with eosinophilia (EAE), known as Gleich’s syndrome, is a rare disease characterized by hypereosinophilia and recurrent episodes of angioedema (AE), urticaria, weight gain, and fever, that occur at periodical intervals [1,2,3,4]
EAE patients usually show homogeneous clinical presentations and wellcharacterized laboratory findings; these considerations suggest that EAE should be considered a distinct eosinophilic disease that is likely caused by a common pathogenetic mechanism in affected subjects [3]
The exact etiology of EAE is still unclear. Both eosinophils and abnormalities of cytokine homeostasis seem to play a pivotal role in the pathogenesis of the disease, published maps and institutional affiliations

Summary

Case Report

A thirty-two-year-old female accessed our Immunology Clinic reporting a history of cyclical attacks of AE, urticaria, and periodic weight gain (up to 10 kg) during the previous two years that caused several visits to the Emergency Room. Bone marrow aspiration and biopsy were performed to rule out any associated malignancy, which showed only an increase in eosinophilic precursors and mild T cell (CD5+ , CD20- ) infiltrate, but no atypical cells. The patient was diagnosed with EAE and she was treated with high doses of oral glucocorticoids (Prednisone 75 mg/day for two weeks) with symptoms resolution and normalization of eosinophil counts. Multiple small reactive lymphadenopathies were al tected at examination of the cervical and inguinal regions and at the Po. AEC: absolute eosinophil count at the onset; ANC: absolute neutrophil count at the onset; ANA: anti-nuclear of 15 antibodies; ANCA: anti-neutrophil cytoplasmic antibodies; CPR: C-reactive protein; CT: computed 5tomography; ESR: erythrocyte sedimentation rate; GI: gastrointestinal. Nancy, which showed only an increase in eosinophilic precursors and mild T cell (CD5+, CD20‐) infiltrate, but no atypical cells

Research Method

Episodic Angioedema with Hypereosinophilia Clinical and Laboratory Features

Findings

Differential Diagnosis