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.     

Retrospective analysis of hypereosinophilic dermatitis

目的:进一步认识嗜酸性粒细胞增多性皮炎(HED)的临床特点.方法:回顾性分析59例嗜酸性粒细胞增多综合征(HES)患者的临床资料,将仅出现血液系统异常及皮肤损害的患者设定为研究组(HED组),其他类型的HES患者设定为对照组.对比分析两组患者的临床表现、实验室及辅助检查结果、治疗方案和临床疗效.结果:HED组患者平均发病年龄为(64.71±11.43)岁,明显晚于对照组[(33.65±13.19)岁](P ＜ 0.01),其首发症状均表现为瘙痒性皮炎.该组患者对治疗的反应存在较大的个体差异,未发现死亡病例.结论:HED可能为HES的一个预后较好的亚型,多见于老年男性患者.该病患者宜尽量明确嗜酸性粒细胞增多的原因,以利于确定个体化治疗方案而控制疾病发展.     

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     

嗜酸性粒细胞增多综合征

嗜酸性粒细胞增多综合征(hypereosinophilic syn-dromes,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。由于HES常伴皮肤表现,国内散发的病例报告逐渐增多,故对该综合征作一综述,以增加了解。1HES的诊断HES是持续的EC增多同时伴有因EC浸润或继发于EC损害组织所致的器官/系统功能障碍的综合征。1975年Chusid等[1]提出特发性HES的诊断标准,目前仍在使用:(1)外周血EC绝对计数>1.5×109/L,并持续6个月以上;或者少于6个月但伴有器官受损的证据;(2)除外其他原因引起的E…     

嗜酸性粒细胞增多性皮炎30例临床分析

目的:探讨嗜酸性粒细胞增多性皮炎(HED)的诊断及治疗策略。方法:对2011年1月—2015年8月该院30例HED住院患者的临床资料进行回顾性分析并随访。结果:30例患者男女比为1.73∶1,平均年龄55.2岁。皮损特点为泛发性及多形性损害,伴剧痒。血常规和骨髓细胞学均显示嗜酸性粒细胞增多。皮损组织病理检查示真皮层见以嗜酸性粒细胞及淋巴细胞为主的炎性细胞浸润。在口服抗组胺药物基础上,予单用小、中剂量糖皮质激素或联合免疫抑制剂治疗效果最佳。结论:糖皮质激素联合抗组胺药为HED治疗首选药物,联合应用免疫抑制剂有协同作用。     

嗜酸性粒细胞增多性皮炎12例临床分析

嗜酸性粒细胞增多性皮炎（HED）是一组病因不明,血及骨髓嗜酸粒细胞持续性增多,组织中嗜酸粒细胞浸润为特征,仅累及皮肤,未累及其他器官的一组疾病,属于嗜酸性粒细胞增多综合征的一种亚型[1],该病皮疹表现无特异性,故临床上易误诊及漏诊。现将我院收治的12例HED的临床资料回顾性分析。     

嗜酸粒细胞增多性皮炎16例临床分析

对1994年4月~2005年4月在上海华山医院住院的16例嗜酸粒细胞增多性皮炎(HED)患者的临床表现、实验室检查及辅助检查结果、治疗方法进行统计分析。HED是嗜酸粒细胞增多综合征(IHS)病谱的良性一端,早期诊断和治疗是十分重要的。皮肤损害特点是多样性、泛发性和反复发作性,联合使用糖皮质激素和免疫抑制剂常获显著效果。     

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.     

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％)....     

嗜酸性粒细胞和高嗜酸性粒细胞综合征

近年来对高嗜酸性粒细胞综合征(HES)的研究已取得不少进展.这些研究的重点主要集中在嗜酸性粒细胞(Eos)内成分的毒性、HES发生机理和病理改变、临床表现方面.综述如下.一、Eos及其与HES的关系Eos的生成很可能受单一基因控制,Eos存骨髓内的形成还受T淋巴细胞制约.个别家族易发生Eos明显增多的病种.Eos的特征性颗粒内有一电子致密的核和电子可穿透的基质,外有膜包绕.核由     

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.     

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.     

Idiopathic hypereosinophilic syndrome associated with cutaneous infarction and deep venous thrombosis

We report a case of idiopathic hypereosinophilic syndrome (HES) presenting with cutaneous infarction and subsequent extensive deep vein thrombosis. The eosinophilia improved dramatically with systemic corticosteroid therapy. A variety of skin disorders have been associated with HES, although there are no previous reports of HES associated with cutaneous infarction. HES is a rare disorder characterized by a sustained overproduction of eosinophils and multisystem disease. The aetiology of the eosinophilia remains uncertain but clonal populations of abnormal T-cells producing interleukin 5 may be implicated.     

Dermal endothelial cells express eotaxin in hypereosinophilic syndrome

We describe a patient with hypereosinophilic syndrome, who had severe cutaneous eruptions. Immunohistochemical study revealed that dermal endothelial cells in the lesional skin of this patient expressed eotaxin, which indicates eotaxin plays a part in inducing eosinophil migration into cutaneous tissue in hypereosinophilic syndrome.     

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.     

Cutaneous microthrombi: a histologic clue to the diagnosis of hypereosinophilic syndrome

Hypereosinophilic syndrome is a rare systemic disease that frequently has cutaneous lesions. Past reviews of the histopathology of the skin lesions have revealed a nonspecific dermal infiltrate with occasional eosinophils as a constant finding. A case of hypereosinophilic syndrome with cutaneous manifestations, in which the skin biopsy demonstrated multiple cutaneous microthrombi, is reported. The diagnostic and prognostic significance of this finding is discussed.     

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。     

Idiopathic hypereosinophilic syndrome and bullous pemphigoid

BACKGROUND: We report the case of bullous pemphigoid associated with hypereosinophilic syndrome. This association has only been report only once in the literature.CASE REPORT: A 58 year-old man was admitted for a surinfected, pruriginous and generalized bullous dermatosis. Physical examination revealed bronchial rales. The cutaneous histology showed a junctional and intradermic cleavage associated with massive dermal infiltration by eosinophils. The diagnosis of a pemphigoid was confirmed by immunology. In parallel, the idiopathic hypereosinophilic syndrome was evoked in view of persisting hypereosinophilia without detected aetiology and associated with pulmonary infiltration. The skin lesions disappeared under symptomatic treatment, but the patient was rehospitalized 4 months later for severe relapse of dermatosis associated with medullary infiltration by eosinophils. Oral corticosteroid therapy gave spectacular results on both skin and blood formula.DISCUSSION: This association is rare, but seems to be related by the same immunological factors highlighting eosinophils. In addition, the presence of hypereosinophilic syndrome gives the pemphigoid some particular clinical, histological and evolutive characteristics.     

Eosinophilic dermatoses

Morphological and functional properties of the eosinophilic granulocyte (e. G.) feature this haematopoietic stem cell-derived cell type as an important cellular component of defense mechanisms, immunologic reactions and proinflammatory/neoplastic processes. Over the last decade significant advances of the molecular pathophysiology of eosinophilic disorders enable increasingly the distinction between the more common reactive (secondary) and clonal eosinophilia including the hypereosinophilic syndrome. This review features a comprehensive clinical summary of dermatological disorders that are frequently associated with transient or persistent eosinophilia belonging to the reactive eosinophilia. The hypereosinophilic syndrome is a subset of idiopathic eosinophilia frequently associated with major tissue targets as skin, heart and others. Therefore, the hypereosinophilic syndrome has to be considered as important differential diagnosis. Most recently, the identification of selective targets (e. g. IL-5, CD52) has translated into therapeutic approaches with monoclonal antibodies such as mepolizumab, alemtuzumab or SCH55700.     

Wells' syndrome associated with idiopathic hypereosinophilic syndrome

Wells’ syndrome, or eosinophilic cellulitis, is characterized by recurrent cutaneous swellings which resemble acute bacterial cellulitis, and by distinctive histopathological changes. Skin lesions show dermal eosinophilic infiltration and the characteristic‘flame figures, which are composed of eosinophil major protein deposited on collagen bundles. The idiopathic hypereosinophilic syndrome is a multisystem disease with a high mortality rate. It is characterized by peripheral blood eosinophilia and eosinophilic infiltration of many organs, including the skin. The most common skin lesions are pruritic maculopapules and nodules over the trunk and limbs, with urticaria and angio-oedema. In contrast to Wells’ syndrome, the pathology of these skin lesions is non-specific with variable eosinophil infiltration. We report overlapping clinical and histopathological findings characteristic of both syndromes in one patient. Our data favour the hypothesis that both syndromes represent an abnormal eosinophilic response to a variety of underlying diseases or causative agents and thus are different expressions of one disease entity linked to theimmunobiology of eosinophils.