Episodic angioedema associated with eosinophilia*
نویسندگان
چکیده
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.
منابع مشابه
Non-episodic angioedema associated with eosinophilia.
To cite: Shikino K, Hirose Y, Nakagawa S, et al. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2016217428 DESCRIPTION A 26-year-old woman presented with a 1-week history of peripheral oedema, fever and polyarthralgia. Physical examination revealed symmetrical nonpitting oedema of her hands and legs (figure 1). Her medical history was unremarkable. Laboratory fin...
متن کاملA dramatic case of non-episodic angioedema with eosinophilia
Gleich Syndrome, also known as episodic angioedema with eosinophilia, was first described in 1984 by Gleich et al as a rare cause of hypereosinophilia. Angioedema with eosinophilia can be classified into 2 types: episodic or non-episodic. We present a dramatic case of non-episodic angioedema with eosinophilia in a young male. A 34 year old construction worker, presented to the emergency departm...
متن کاملEpisodic angioedema with eosinophilia (Gleich’s syndrome) is a multilineage cell cycling disorder
Episodic Angioedema with Eosinophilia (Gleich’s Syndrome) is a rare disorder characterized by episodes of angioedema and eosinophilia that occur at monthly intervals and resolve spontaneously without therapy. Despite the striking periodicity of this disorder, its similarity to other cyclic hematopoietic disorders with multilineage involvement has not been assessed. To characterize the involveme...
متن کاملEpisodic angioedema with eosinophilia (Gleich syndrome) is a multilineage cell cycling disorder.
Episodic angioedema with eosinophilia (Gleich syndrome) is a rare disorder characterized by episodes of angioedema and eosinophilia that occur at monthly intervals and resolve spontaneously without therapy. Despite the striking periodicity of this disorder, its similarity to other cyclic hematopoietic disorders with multilineage involvement has not been assessed. To characterize the involvement...
متن کاملElevated serum levels of interleukin-5 in patients with the syndrome of episodic angioedema and eosinophilia.
The syndrome of episodic angioedema and eosinophilia is characterized by cyclic edema, marked peripheral blood eosinophilia, and eosinophil degranulation in the dermis. Using a sensitive immunoenzymetric method, we measured serum interleukin (IL)-5 levels in four patients with this syndrome. We also determined the percentage of activated T cells in the peripheral blood of a new patient before a...
متن کاملA case report of nonepisodic angioedema with eosinophilia in a Korean patient and a review of the Korean literature
Episodic angioedema with eosinophilia (EAE) is characterized by recurrent angioedema, peripheral eosinophilia, elevated serum IgM, fever, weight gain, and a benign course lacking any internal organ involvement. Dozens of cases of the nonepisodic variant (NEAE), which is limited to a single attack, have been reported in Japan. These NEAE cases normally have been less severe than the episodic typ...
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