Clinical Characteristics of Angioedema With Eosinophilia

Cho, Hyo Jung; Yoo, Hye-Soo; Kim, Mi-Ae; Shin, Yoo Seob; Ye, Young-Min; Nahm, Dong-Ho; Kim, Joo Hee; Choi, Jeong‐Hee; Park, Sun Young; Park, Hae‐Sim

doi:10.4168/aair.2014.6.4.362

Cited by 20 publications

(19 citation statements)

References 25 publications

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“…In contrast, nonepisodic angioedema with eosinophilia (NEAE), proposed by Chikama et al in 1998, 2 is more common in Japan 4,5 and Korea, 6,7 with almost all cases occurring among young adult females. NEAE is also clinically diagnosed based on its characteristic clinical presentation, including a monophasic episode, angioedema of the extremities, a nonfebrile state, eosinophilia, normal serum levels of IgM, and lack of involvement of the internal organs.…”

Section: Introductionmentioning

confidence: 76%

Histological Classification and Differential Diagnosis of Nonepisodic Angioedema With Eosinophilia

Goto

Ishizaki

Tanese

et al. 2016

The American Journal of Dermatopathology

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Nonepisodic angioedema with eosinophilia (NEAE) is a rare condition characterized with monoepisodic angioedema, a nonfebrile state, eosinophilia, normal serum IgM levels, and lack of internal organ involvement. The histology of this disease is not yet well known. The purpose of this study was to characterize the histopathologic features of NEAE. Twelve cases of clinically confirmed NEAE were retrieved from 6 institutions, and these cases were reviewed regarding the clinical data and histopathology, particularly regarding granulomatous lesions. The authors demonstrated that the histology of NEAE can be classified into 3 patterns that of eosinophilic granulomatous panniculitis (7/12 cases), eosinophilic dermatitis without granuloma formation (3/12 cases), and invisible dermatosis (2/12 cases). Six of the 7 granulomatous cases showed the characteristic eosinophilic granulomatous lesions containing individual necrotic adipocytes with membranous fat changes, which could be a differential clue to the diagnosis of NEAE. Review of the previously reported cases (n = 37) revealed that the histological classification could be adaptable to these reported cases. The authors should recognize the histological variation of NEAE and distinguish it from the histological mimickers, including eosinophilic granulomatosis with polyangiitis, erythema nodosum, hypereosinophilic syndrome, and episodic angioedema with eosinophilia.

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Section: Introductionmentioning

confidence: 76%

Histological Classification and Differential Diagnosis of Nonepisodic Angioedema With Eosinophilia

Goto

Ishizaki

Tanese

et al. 2016

The American Journal of Dermatopathology

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“…Prednisone was the most frequently prescribed agent with doses ranging from 5 to 60 mg/day depending on the frequency and severity of the exacerbations. The 20 mg dosage proved to be effective most of the time, but in cases of incomplete clinical response, administration of higher doses proved to be effective, 30 and omalizumab 29 and busulfan 19 were used as cortico‐sparing agents in some cases. The use of antihistamines was controversial, 25 but desloratadine alone resulted in complete remission in one patient 27 .…”

Section: Trt and Prognosismentioning

confidence: 99%

Gleich syndrome: a systematic review

et al. 2020

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Characterized chiefly by hypereosinophilia and angioedema, Gleich syndrome is a rare disorder with unclear clinical and therapeutic findings. Other symptoms include increased IgM levels, weight gain, fever, and urticaria. Herein we review Gleich syndrome and assess clinical features, epidemiology, and treatment options. Thirty-two articles including case reports or case series of eosinophilic angioedema and Gleich syndrome were included. Data regarding patient age, gender, and history, clinical and biological manifestations, and treatment protocols were recorded. The most common clinical findings include recurrent or non-recurrent angioedema, fever, urticaria, weight gain, blood eosinophilia, and elevated immunoglobulin levels. Corticosteroid therapy is the mainstay of treatment. Gleich syndrome is a distinctive hypereosinophilic entity with a benign course and good response to systemic corticosteroids. More studies are needed to evaluate the pathophysiology of this syndrome and lead to better therapeutic options.

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“…37 the non-episodic type. 38 Small case series of NEAE with polyarthralgia have also been reported. 39,40 Well's syndrome, also known as eosinophilic cellulitis, may present with a swollen limb and a myriad of cutaneous lesions including plaques, papules, nodules and bullae, resembling bacterial cellulitis.…”

Section: Limbsmentioning

confidence: 99%

“…Majority of the study subjects were female withthe non-episodic type 38. Primary lymphoedema is a recognized developmental anomaly seen in Turner syndrome.…”

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confidence: 99%

Angioedema Masqueraders

Fok

Katelaris

2019

Clin Experimental Allergy

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Angioedema is a common reason for referral to immunology and allergy specialists.Not all cases are in fact angioedema. There are many conditions that may mimic its appearance, resulting in misdiagnosis. This may happen when a clinician is unfamiliar with conditions resembling angioedema or when there is a low index of clinical suspicion. In this article, we explore a list of differential diagnoses based on body parts, including the lips, the limbs, periorbital tissues, the face, epiglottis and uvula, as well as the genitalia, that may pose as a masquerader even to an experienced eye. K E Y W O R D Sallergic contact dermatitis, angioedema, angioedema masqueraders, angioedema mimics, cheilitis granulomatosa, clinical immunology

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Clinical Characteristics of Angioedema With Eosinophilia

Cited by 20 publications

References 25 publications

Histological Classification and Differential Diagnosis of Nonepisodic Angioedema With Eosinophilia

Histological Classification and Differential Diagnosis of Nonepisodic Angioedema With Eosinophilia

Gleich syndrome: a systematic review

Angioedema Masqueraders

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