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Episodic angioedema associated with eosinophilia

We report a 12-year-old girl who presented with recurrent angioedema on the face, trunk, and extremities, and concomitant marked weight gain for 5 years. During the episode, her white blood cell count increased to 47.7×109/L with 89.9% eosinophils, followed by elevated serum level of IL-5, IgE, IgM,...

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Autores principales: Liu, Fang, Hu, Wenxing, Liu, Haibo, Zhang, Min, Sang, Hong
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Sociedade Brasileira de Dermatologia 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5595603/
https://www.ncbi.nlm.nih.gov/pubmed/28954105
http://dx.doi.org/10.1590/abd1806-4841.20174351
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author Liu, Fang
Hu, Wenxing
Liu, Haibo
Zhang, Min
Sang, Hong
author_facet Liu, Fang
Hu, Wenxing
Liu, Haibo
Zhang, Min
Sang, Hong
author_sort Liu, Fang
collection PubMed
description We report a 12-year-old girl who presented with recurrent angioedema on the face, trunk, and extremities, and concomitant marked weight gain for 5 years. During the episode, her white blood cell count increased to 47.7×109/L with 89.9% eosinophils, followed by elevated serum level of IL-5, IgE, IgM, and LDH. Histopathology showed perivascular eosinophilic infiltration and diffuse eosinophilic infiltration throughout the dermis. Possible causes of hypereosinophilia and eosinophilic infiltration of vital organs were ruled out. We also tested the FIP1L1/PDGFRa and ETV6/PDGFRb fusion gene to exclude the possibility of myeloid and lymphatic vessel neoplasms. The patient was treated with methylprednisolone and discharged with an oral prednisolone taper, which resulted in complete remission of the edema and normalization of peripheral blood eosinophil count, serum IL-5 level, IgE, IgM, and LDH.
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spelling pubmed-55956032017-09-19 Episodic angioedema associated with eosinophilia Liu, Fang Hu, Wenxing Liu, Haibo Zhang, Min Sang, Hong An Bras Dermatol Case Report We report a 12-year-old girl who presented with recurrent angioedema on the face, trunk, and extremities, and concomitant marked weight gain for 5 years. During the episode, her white blood cell count increased to 47.7×109/L with 89.9% eosinophils, followed by elevated serum level of IL-5, IgE, IgM, and LDH. Histopathology showed perivascular eosinophilic infiltration and diffuse eosinophilic infiltration throughout the dermis. Possible causes of hypereosinophilia and eosinophilic infiltration of vital organs were ruled out. We also tested the FIP1L1/PDGFRa and ETV6/PDGFRb fusion gene to exclude the possibility of myeloid and lymphatic vessel neoplasms. The patient was treated with methylprednisolone and discharged with an oral prednisolone taper, which resulted in complete remission of the edema and normalization of peripheral blood eosinophil count, serum IL-5 level, IgE, IgM, and LDH. Sociedade Brasileira de Dermatologia 2017 /pmc/articles/PMC5595603/ /pubmed/28954105 http://dx.doi.org/10.1590/abd1806-4841.20174351 Text en http://creativecommons.org/licenses/by-nc-nd/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial No Derivative License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium provided the original work is properly cited and the work is not changed in any way.
spellingShingle Case Report
Liu, Fang
Hu, Wenxing
Liu, Haibo
Zhang, Min
Sang, Hong
Episodic angioedema associated with eosinophilia
title Episodic angioedema associated with eosinophilia
title_full Episodic angioedema associated with eosinophilia
title_fullStr Episodic angioedema associated with eosinophilia
title_full_unstemmed Episodic angioedema associated with eosinophilia
title_short Episodic angioedema associated with eosinophilia
title_sort episodic angioedema associated with eosinophilia
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5595603/
https://www.ncbi.nlm.nih.gov/pubmed/28954105
http://dx.doi.org/10.1590/abd1806-4841.20174351
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AT sanghong episodicangioedemaassociatedwitheosinophilia