Episodic angioedema associated with eosinophilia

Liu, Fang; Hu, Wen-xing; Li, Haibo; Zhang, Min; Sang, Hong

doi:10.1590/abd1806-4841.20174351

Cited by 9 publications

(8 citation statements)

References 7 publications

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“…Fifteen children (10 males, median age 10 years [range 2.5‐17]) with Gleich syndrome living in Europe, Asia, or the United States were identified (Table ) . The median time between the first angioedema flare and the diagnosis of EAE was 5 years (data available for n = 11 patients).…”

Section: Resultsmentioning

confidence: 99%

Episodic angioedema with eosinophilia (Gleich syndrome) in children: A clinical review

Bertrand

Boccara

Filhon

et al. 2019

Pediatric Allergy Immunology

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Background Episodic angioedema with eosinophilia (EAE, Gleich syndrome) is a rare disease, consisting of recurrent angioedema with hypereosinophilia and frequent increased serum immunoglobulin M levels. Less than 100 patients have been reported, mainly adults, sometimes with underlying lymphocytic variant of hypereosinophilic syndrome (HESL). The aim of this study was to identify and describe pediatric cases. Methods We performed a retrospective study of all pediatric cases of EAE referred within the French National Referral Center for Hypereosinophilic Syndrome (CEREO). Next, the PRISMA guidelines were applied in order to perform a systematic review (data sources: PubMed, Web of Science). Results Among the two reported and 15 previously published cases of EAE occurring in children, the main clinical findings mimicked those of adults, including recurrent angioedema, hives, and weight gain. The median time between the first angioedema flare and the diagnosis of EAE was 5 years in published cases. Hypereosinophilia was constant, usually worsening with each attack, but seldom disappeared between flares. Total IgM serum levels were elevated in 16 patients. Four children had evidence of abnormal CD3‐CD4+ T cells. First‐line therapy relied on oral corticosteroids in all patients, and further lines (used in five patients) included interferon‐α, methotrexate, and cyclosporin. Two children developed eosinophilic myocarditis during follow‐up. Conclusion Pediatricians should be aware that EAE is a diagnosis to consider in children. T‐cell immunophenotyping is warranted in this setting. Prognosis seems fair, yet eosinophil‐related organ damage may occur in patients with persistent eosinophilia.

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Section: Resultsmentioning

confidence: 99%

Episodic angioedema with eosinophilia (Gleich syndrome) in children: A clinical review

Bertrand

Boccara

Filhon

et al. 2019

Pediatric Allergy Immunology

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“…Gleich's syndrome, also known as episodic angioedema syndrome with eosinophilia, is a rare disorder of unclear etiology, although a Th2 pathway imbalance with increased interleukins IL5, IL6, IL13 and eosinophils during flares has been described. 60,61 Clinical features include recurring episodes of idiopathic angioedema associated with eosinophilia and elevated serum immunoglobulin IgM levels without systemic organ involvement. 4 This entity should be distinguished from classical angioedema by the presence of specific laboratory features (hypereosinophilia and increased IgM levels) together with other characteristic clinical manifestations (weight gain, fever, and urticaria).…”

Section: Gleich's Syndromementioning

confidence: 99%

If not angioedema, what is it? Diagnostic approach to facial edema

Velasco‐Amador,

Prados‐Carmona,

Navarro‐Triviño

2024

J Deutsche Derma Gesell

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SummaryFacial edema is a relatively frequent clinical presentation encountered in patients seen in allergology and dermatology clinics. The differential diagnosis is broad, and sometimes the definitive diagnosis can be a challenge for the clinician. Facial angioedema itself encompasses different etiopathologies (histaminergic, bradykinergic, etc.) that must be distinguished from other causes of facial edema, such as allergic contact dermatitis, granulomatous conditions, inflammatory causes, infections, neoplasms or paraneoplastic syndromes, autoimmune diseases, among other entities hereby referred as miscellanea. A proper diagnostic approach is essential to order the appropriate tests, as well as to prescribe a targeted treatment. This review focuses on entities that present with facial edema and summarize their characteristic clinical features.

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“…A wide range of total eosinophil counts can be found at presentation (from 1000 to 23,400 cells/mm 3 ) ( 8 ). In contrast to hypereosinophilic syndromes, organ dysfunction is not observed among patients with EAE ( 9 ). Indeed, an extensive diagnostic workup failed to find eosinophilic infiltration and organ dysfunction in our patient.…”

Section: Case Reportmentioning

confidence: 99%

Episodic angioedema with eosinophilia (Gleich's syndrome) associated with urticarial vasculitis: a coincidence or a novel clinical entity?

Basso

Bizinoto

Limone

et al. 2021

Braz J Med Biol Res

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Episodic angioedema with eosinophilia (EAE) is a rare condition characterized by recurrent attacks of angioedema and urticaria accompanied by a marked elevation of peripheral eosinophil count. We report the case of a young female patient diagnosed with EAE associated with urticarial vasculitis. A 40-year-old female patient was admitted to our institution due to recurrent episodes of cheek and eyelid angioedema in the previous year. Episodes of facial angioedema lasted for two months with spontaneous remission afterwards. In addition, she presented pruritic and painful skin eruptions of erythematous circles, which persisted for longer than 24 h, that were palpable, somewhat purplish, and more pronounced on the face, arms, and trunk. Laboratory investigation showed a sustained elevation of white cell counts with marked eosinophilia. Serum IgM, IgE, and IgA were normal; IgG was slightly elevated. C1-esterase inhibitor and tryptase test were normal. Reverse transcriptase-polymerase chain reaction was performed for detection of FIP1L1-PDGFRA and BCR-ABL rearrangements. None of these alterations were found. Skin biopsies were suggestive of urticarial vasculitis. The patient was submitted to esophagogastroduodenoscopy, which showed mild chronic gastritis, with no eosinophilic infiltration. Cardiac dimensions and function were normal. Abdominal ultrasound and total body CT-scan failed to show lymphadenopathy, organomegaly, and tumors. We report the first case of association between episodic angioedema with eosinophilia and urticarial vasculitis. It is possible that both conditions share a physiopathological mechanism, suggesting that it is not just a chance association.

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Episodic angioedema associated with eosinophilia

Cited by 9 publications

References 7 publications

Episodic angioedema with eosinophilia (Gleich syndrome) in children: A clinical review

Episodic angioedema with eosinophilia (Gleich syndrome) in children: A clinical review

If not angioedema, what is it? Diagnostic approach to facial edema

Episodic angioedema with eosinophilia (Gleich's syndrome) associated with urticarial vasculitis: a coincidence or a novel clinical entity?

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