嗜酸性粒细胞增多综合征1例

患者女,53岁。反复咳嗽、咳痰1年,全身起疹伴瘙痒2个月入院。入院检查外周血和骨髓嗜酸性粒细胞明显增多,组织病理改变符合嗜酸性粒细胞改变。诊断:嗜酸性粒细胞增多综合征。给予糖皮质激素、抗感染和支持对症等治疗14d后出院。现随访中。     

嗜酸性粒细胞增多综合征1例并文献复习

正嗜酸性粒细胞增多综合征(Hypereosinophilic Syndrome,HES)是以血及骨髓嗜酸性粒细胞增多,组织中嗜酸性粒细胞浸润为特征的一类异质性疾病谱[1]。多累及皮肤、心血管、呼吸、消化及血液系统,并出现相关症状及体征。嗜酸性粒细胞增多性皮炎和慢性嗜酸性粒细胞性白血病分别为该疾病谱的良性端及恶性端,嗜酸性粒细胞增多性皮炎(Hypereosinophilic Dermatitis,HED),是一种仅侵犯皮肤而无系统性损害的亚型,皮疹多型、泛发伴剧烈瘙痒,预后尚可,该定义由Nir及Westfried     

嗜酸性粒细胞增多性皮炎的治疗进展

嗜酸性粒细胞增多综合征（hypereosinophilic syndrome,HES）是一组病因不明,以血液和/或骨髓嗜酸性粒细胞（eosinophil cell,EC）持续增多,组织中大量EC浸润为特征的疾病.而嗜酸性粒细胞增多性皮炎（HED）是嗜酸性粒细胞增多综合征（HES）的轻型或此疾病谱的良性端.它是嗜酸性粒细胞增多引起的皮肤病.近年来,临床上报道的HED病例越来越多.对其病因和发病机制的研究也已经取得很大进展,治疗上也取得一些的进展.     

嗜酸性蜂窝织炎

嗜酸性蜂窝织炎(Eosinophilic Cellulitis)是一种少见的、有独特的临床(开始似蜂窝织炎样、随后变为硬斑样的单发或多发性皮损)和组织病理改变(真皮有明显的嗜酸性粒细胞浸润和有火焰样图形的炎症性肉芽肿)特征,并伴外周血嗜酸性粒细胞增多的复发性皮肤病。本病首先由Wells在1971年以“伴有嗜酸性粒细胞增多的复发性肉芽肿性皮炎”(Recurrent Gnanulomatous Derma—     

嗜酸性粒细胞增多综合征1例

报告1例嗜酸性粒细胞增多综合征。患者男，57岁。四肢反复出现丘疹、血疱伴间断发热半年入院。既往有慢性结肠炎病史20年。入院检查外周血和骨髓嗜酸性粒细胞明显增多，淋巴结组织病理改变符合嗜酸性粒细胞白血病淋巴结改变给予糖皮质激素、抗感染和支持对症等治疗18d后出院。出院后1个月死于心力衰竭。     

嗜酸性粒细胞增多综合征的诊断与治疗

嗜酸性粒细胞增多综合征（HES）是一类与嗜酸粒细胞相关并具有共同临床特点的谱性疾病.疾病的一端为病程良性的嗜酸性粒细胞增多性皮炎,仅累及皮肤,无器官或系统受累,预后较好;疾病的另一端为慢性嗜酸性粒细胞白血病或淋巴瘤,可以累及全身多个器官、系统,出现较严重的临床症状,甚至导致死亡,预后差.该病1968年由Hardy和Anderson首先提出,认为HES是病因不明、血液及骨髓中嗜酸性粒细胞（EOS）持续增多,组织中嗜酸性粒细胞（EOS）浸润为特征的一类疾病.     

泛发性白癜风合并嗜酸性粒细胞增多性皮炎1例

白癜风是临床上较为常见的一种皮肤病，可合并多种其他疾病，嗜酸性粒细胞增多性皮炎国内已有报道，但白癜风合并嗜酸性粒细胞增多性皮炎者尚未见报道，现将们所见1例报道如下。     

卡介菌多糖核酸等治疗嗜酸性粒细胞增多性皮炎2例

2例嗜酸性粒细胞增多性皮炎患者予肌注卡介菌多糖核酸注射液2ml/d,同时口服抗胺药物,3～6周后皮损基本消退,外周血嗜酸性粒细胞计数恢复正常,无明显不良反应。     

嗜酸性粒细胞增多性皮炎11例临床分析

目的探讨嗜酸性粒细胞增多性皮炎的临床特点。方法回顾性分析嗜酸性粒细胞增多性皮炎患者的临床资料。结果11例患者中男8例、女3例，平均年龄（51．3±16．7）岁，病程7个月～17年。皮疹泛发、多形、剧痒，伴有一定系统损害。糖皮质激素治疗有效，甘草制剂有一定疗效，部分患者多种治疗抵抗。结论嗜酸性粒细胞增多性皮炎患者多发于男性，甘草制剂有一定疗效。     

以红皮病为主要表现的嗜酸性粒细胞增多性皮炎1例

报告1例以红皮病为主要临床表现的嗜酸性粒细胞增多性皮炎。患者男,78岁。全身红斑、丘疹反复30年,泛发红皮8个月。全身皮肤弥漫潮红,粗糙,部分呈苔藓样改变,可见散在圆顶丘疹,色素沉着,细白脱屑,头皮为甚。外周血EOS明显升高超过半年,并随皮疹反复而增多,未发现其他器官损害,结合皮肤组织病理及骨髓象检查,诊断为嗜酸性粒细胞增多性皮炎。     

嗜酸粒细胞增多性皮炎9例临床分析

目的：探讨嗜酸粒细胞增多性皮炎的临床特征。方法：对1996～2006年间收住院的9例嗜酸粒细胞增多性皮炎患者的临床资料进行回顾性分析。结果：嗜酸粒细胞增多性皮炎以中老年男性为主，病程反复迁延，皮损泛发、多形、剧烈瘙痒，以光泽坚实的红色丘疹、浸润肥厚性斑块、浸润水肿性肿胀颇为特殊。结论：嗜酸粒细胞增多性皮炎以皮肤损害为主，同时存在着内脏器官受累的可能。     

Hypereosinophile Dermatitis

BACKGROUND: The idiopathic hypereosinophilic syndrome is a rare systemic disease characterized by blood and tissue eosinophilia of unknown etiology, in which multiple organs may be affected. In hypereosinophilic dermatitis the only affected organ besides the blood is the skin. PATIENTS: We present a series of seven patients with hypereosinophilic dermatitis who were treated in our hospital between 2002 and 2003. RESULTS: All patients initially showed characteristic, therapy-resistant, polymorphic skin lesions, presenting with a combination of erythematous, pruritic and urticarial papules and plaques. All had blood eosinophilia without evidence of allergic, parasitic or other causes. The histology showed tissue eosinophilia only in half of the cases; the other histological findings were non-specific. We observed a good response to therapy with systemic corticosteroids, dapsone and light therapy, applied as UVA-1 irradiation or as shower photochemotherapy. CONCLUSIONS: The diagnosis "hypereosinophilic dermatitis" should be based primarily on the characteristic clinical picture together with "idiopathic" peripheral eosinophilia, whereas the histological findings are not always indicative. Because of the multiplicity of possible differential diagnoses and the often non-revealing histology, we assume that the diagnosis "hypereosinophilic dermatitis" is often overlooked.     

Recurrent angioedema with eosinophilic dermatitis--minus variants of the hypereosinophilic syndrome

Recurrent angio-oedema with eosinophilic dermatitis is characterized by the following symptoms: persistent hypereosinophilic, episodes of angio-oedema, urticarial papular and papulo-pustular exanthema, episodes of fever, as well as increased IgE and IgM levels. Eosinophilic dermatitis with subcorneal pustulation can be seen on histological examination. This disease should be distinguished from the classical hypereosinophilic syndrome because of the absence of internal symptoms and because the prognosis is apparently favourable. Internal corticosteroids are the therapy of choice.     

嗜酸粒细胞增多综合征的治疗进展

嗜酸粒细胞增多综合征是病因不明,以骨髓、外周血和组织中嗜酸粒细胞增多为特点,并累及多器官的一组疾病,仅累及皮肤者称为嗜酸粒细胞增多性皮炎.传统药物如糖皮质激素、羟基脲、环孢素等均为非特异性作用药物,长期服用不良反应明显.近年发现,该病与嗜酸系融合基因或异常T淋巴细胞克隆有关,酪氨酸激酶抑制剂、干扰素α、白介素-5单克隆抗体等对该病有特异性疗效.     

Detection of HSV-specific DNA in biopsy tissue of patients with erythema multiforme by polymerase chain reaction

Formalin-fixed paraffin-embedded skin biopsies of lesions of erythema multiforme (EM) from 32 patients and 13 controls were examined for the presence of herpes simplex virus (HSV) by polymerase chain reaction (PCR) and for histological findings by direct immunofluorescence and staining with haematoxylin and eosin. HSV-specific DNA was detected in 23 (72%) patients. A history of recurrent skin rash was present in 59% of the PCR-positive cases, while 55% had had suspected HSV infections. Only two PCR-positive specimens were found in patients without a history of recurrent rash and/or previous oral lesions. One biopsy was positive for HSV by conventional cell cultures. There was no significant difference in histology between HSV-related and HSV-negative cases of EM. In the 13 control specimens [bullous pemphigoid (3), dermatitis herpetiformis (2), lichen planus (1), aphthous ulcer (1), fixed-drug eruption (1), varicella-zoster (1), hypereosinophilic syndrome (1), photocontact dermatitis (1), contact dermatitis (1), and cellulitis (1)], no HSV-DNA was detected.     

Isolated symptomatic cutaneous disease in hypereosinophilic syndrome

A 41-year-old Phillipino man presented with a 3-year history of a relapsing and remitting generalized chronic pruritic erythematous papular and plaque-like eruption. Investigations showed a persistently elevated eosinophil count. His disease was limited to cutaneous involvement with an absence of demonstrable internal organ involvement, despite extensive investigations and multidisciplinary review. Other causes of eosinophilia were excluded. A diagnosis of idiopathic hypereosinophilic syndrome was made. Our patient's presentation raises a number of issues related to hypereosinophilic syndrome. In particular, relating to managing hypereosinophilic syndrome and the challenge of minimizing therapy side-effects. Our case highlights the considerable morbidity of untreated isolated cutaneous disease, for which he was hospitalized with suicidal ideations. In a minority of reports, skin involvement is the only manifestation of hypereosinophilic syndrome.     

嗜酸粒细胞增多性皮炎五例并文献复习

目的探讨嗜酸粒细胞增多性皮炎（HED）的临床特征。方法对2005年以来收治的5例HED患者的临床资料进行回顾性分析,并复习文献,讨论其临床表现与治疗策略。结果 5例患者平均发病年龄65岁,以中老年人为主。皮损类型以红斑、丘疹、结节为主。3例患者行FIP1L1-PDGFRA融合基因检测阴性。5例患者血清IgE均明显升高。中小剂量糖皮质激素和细胞毒类药物对HED治疗有效。免疫调节剂（α干扰素、卡介菌多糖核酸）和生物制剂具有良好疗效。结论早期诊断、个体化治疗及定期随访对HED管理非常重要。     

Hypereosinophilic dermatitis-like erythema annulare centrifugum in a patient with chronic lymphocytic leukaemia

A 65-year-old male presented with a history of disseminated erythema annulare centrifugum lesions and a high peripheral blood eosinophilia (55%). Histopathology of a lesion revealed a superficial and deep perivascular and interstitial inflammatory eosinophilic infiltrate. The bone marrow aspirate showed a heavy eosinophilic infiltrate. There was no evidence of any other systemic involvement except for bone marrow eosinophilia. The patient had a 6-year history of chronic lymphocytic leukaemia. The disease is in complete remission for the last 3 years. The clinical and histopathological findings in the reported patient correspond to the diagnosis of hypereosinophilic dermatitis. Topical treatment with corticosteroid creams was successful. The cutaneous lesions gradually resolved in 8 weeks, within 7 months the peripheral blood eosinophilia slowly declined from 55% to 7%.     

嗜酸性粒细胞增多性皮炎16例回顾性分析

目的:探讨嗜酸性粒细胞增多性皮炎(HED)的临床特点、治疗方案及后期转归情况,为临床诊断和治疗提供参考.方法:对2014—2019年16例HED住院患者临床资料及实验室检查进行回顾性分析.结果:16例患者中,男女发病比为7:1,皮疹主要表现为红斑、丘疹(约70％),多全身泛发,伴有明显瘙痒.误诊10例(62.50％)....