嗜酸粒细胞增多综合征和嗜酸性皮肤病

嗜酸粒细胞增多综合征是一组以外周血嗜酸粒细胞持续性升高,伴有器官损害或功能不全的疾病,可分为髓系增生性、淋巴细胞性、重叠型、家族性、伴随现象和未分类等临床亚型,其中包括良性和恶性疾病.嗜酸性皮病是指以皮肤组织炎性浸润中存在嗜酸粒细胞为特征的一组皮肤病,常见的有昆虫叮咬、药疹、妊娠性皮病、荨麻疹、血管性水肿、过敏性接触性皮炎和特应性皮炎等,还包括一些具有组织病理特点的疾病,如Well综合征、木村病、嗜酸粒细胞增多性脓疱性毛囊炎、面部肉芽肿等.     

嗜酸粒细胞增多综合征一例

患者男,22岁.因全身皮疹十余年,加重1年伴痒,于2006年7月31日入院,患者10年前不明原因双肘部、双小腿屈侧出现红斑,瘙痒明显,抓后出现糜烂、渗出及浸润,在外院就诊,诊断为湿疹,给予治疗(用药不详),皮疹持续存在.近1年皮疹增多,泛发躯干、四肢,皮肤出现潮红、瘙痒明显,颈部、腋下、股内侧淋巴结肿大.患者父母非近亲结婚,否认家族中有类似疾病患者.     

嗜酸细胞增多综合征1例

嗜酸细胞增多综合征临床上不多见且易误诊,现将所遇一例报告如下。 患者男,12岁。3岁起出现哮喘并迁延不愈。5岁时下肢出现绿豆大皮疹,渐延及全身且伴有剧烈瘙痒。在当地曾两次住院60余天未能确诊,其间服用过强的松、氨苯砜、海群     

嗜酸粒细胞增多综合征12例临床分析

对2003年6月—2010年6月间在武汉市第一医院皮肤科住院的12例嗜酸粒细胞增多综合征患者的临床表现、实验室检查、治疗等临床资料进行回顾性分析。嗜酸粒细胞增多综合征以中老年男性为主,皮疹表现多形,可合并多项实验室检查改变及系统损害,临床易误诊,早期诊断和治疗十分重要。单独或联合使用糖皮质激素常获较显著效果。     

复杂性未定类嗜酸粒细胞增多综合征一例

患者男,41岁,全身泛发红斑、丘疹,偶颜面水肿伴痒1年半,家族中无类似患者.实验室检查:外周血嗜酸粒细胞增多,骨髓活检:嗜酸粒细胞比例增高,多次粪检未见寄生虫卵,血多种寄生虫抗体阴性,心肌酶升高,抗中性粒细胞胞质抗体阴性,FIP1L1/PDGFRα融合基因阴性;心脏彩色超声见轻度三尖瓣和肺动脉瓣关闭不全,右房轻度增大.诊断为复杂性未定类嗜酸粒细胞增多综合征,予泼尼松联合环磷酰胺治疗后,症状控制理想.     

嗜酸粒细胞增多性皮炎的研究进展

嗜酸粒细胞增多性皮炎指一组病因不明、外周血及骨髓中嗜酸粒细胞长期持续增多、仅累及皮肤的一组疾病,具有明显异质性。近年来,该病的发病率有逐年增加的趋势,临床上易发生误诊及漏诊,且缺乏有效的治疗手段。该文旨在总结该病发病机制的进展,以便明确疾病本质,找出有效的治疗对策。     

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。     

嗜酸粒细胞增多性皮炎19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2005-2013年本科收住院的19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。结果共入选19例,其中男12例,女7例;年龄50~81岁,病程均反复迁延不愈,表现为泛发性、多形性、瘙痒性皮炎样皮损。血常规、外周血涂片、骨髓像均显示嗜酸粒细胞增多。皮损组织病理示真皮及皮下组织血管扩张,周围见大量炎症细胞浸润,以嗜酸粒细胞及淋巴细胞为主。结论嗜酸粒细胞增多性皮炎以皮肤损害为主,治疗方面应采用个体化方案。     

以皮肤损害为突出表现的嗜酸粒细胞增多综合征1例

报告1例以皮肤损害为突出表现的嗜酸粒细胞增多综合征。患者男,70岁,反复皮肤瘙痒伴增厚、红色斑块半年,加重1月,同时伴肝、肾损害、脾大。皮损组织病理:真皮水肿,真皮浅深层及血管周围可见以嗜酸粒细胞和单一核细胞为主的浸润,部分血管壁可见嗜酸粒细胞移入,伴嗜酸粒细胞脱颗粒现象。诊断为嗜酸粒细胞增多综合征     

嗜酸粒细胞增多性皮肤病

近年来，对嗜酸粒细胞大分子物质及其生物学作用的研究取得显著进展，深化人们对嗜酸粒细胞增多性疾病的认识和关注。Sade等对100例嗜酸粒细胞增多性疾病住院患者的回顾性分析中发现，哮喘和其他特应性疾病占13％，嗜酸性肺炎10％，肿瘤10％，感染10％，原因不明34％，而特发性嗜酸粒细胞增多综合征、Churg-Strauss综合征、皮肤病、药物过敏和真菌过敏占23％。足见与皮肤病相关疾病占相当大的比例。[第一段]     

A case of hypereosinophilic syndrome

A 22-year-old man developed papules on his legs in November of 2001, which then spread to cover almost his entire body. He was treated with a topical steroid and PUVA therapy at another hospital. The symptoms showed no improvement, and, in February of 2002, he came to our hospital. Examination revealed hypereosinophilia, and, because he had symptoms of organ involvement by the heart, lung, and inguinal lymph nodes as well as the skin, we diagnosed him with idiopathic hypereosinophilic syndrome (HES). The patient was treated with oral prednisolone at a dose of 60 mg/day, and his cutaneous lesions and other organ symptoms improved.     

Hypereosinophilic syndrome as prodrome of bullous pemphigoid:report of a case

Persistent hypereosinophilia, cardiac involvement and a recurrent erythematous-papular pruritic eruption histologically characterized by eosinophilic spongiosis are described in a 77-year-old man. This condition, suggestive of "idiopathic" hypereosinophilic syndrome, represented the prodromic phase of atypical bullous pemphigoid, which manifested 7 months later.     

Case of hypereosinophilic syndrome with cutaneous necrotizing vasculitis

A 25-year-old male presented with pruritic papules over his trunk and extremities. The pruritic eruption had appeared on the arms 5 months before and gradually spread over the trunk. Blood tests showed a white blood cell count of 13 760/microL with 42.2% eosinophils. A skin biopsy from the leg revealed necrotizing vasculitis in the upper dermis with prominent eosinophil infiltration in the dermis and subcutis. Liver dysfunction in accordance with eosinophilia was also seen. The underlying disorder causing the eosinophilia was not detected. We diagnosed this case as hypereosinophilic syndrome. Although systemic corticosteroid therapy using prednisolone was effective, the symptoms recurred during a reduction of prednisolone. The addition of cyclosporine resulted in improvement of his symptoms. Both drugs were successfully tapered without relapsing symptoms. Hypereosinophilic syndrome with cutaneous necrotizing vasculitis is a rare condition, and this case suggests that cyclosporine may be a useful therapeutic adjunct in facilitating steroid tapering.     

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.     

Peripheral Neuropathy Associated withHypereosinophilic Syndrome

The idiopathic hypereosinophilic syndrome (HES) represents a leukoproliferative disorder, characterized by unexplained prolonged eosinophilia (>6 months) and evidence of specific organ damage. So far, the peripheral neuropathy associated with skin manifestations of HES has not been reported in the dermatologic literature although the incidence of peripheral neuropathy after HES ranges from 6~52%. Herein, we report the peripheral neuropathy associated with HES, documented by clinical, histopathological, and electrodiagnostic criteria.     

嗜酸性粒细胞增多性皮炎研究进展

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。骨髓中嗜酸性粒细胞增生后释放入外周血,通过与内皮细胞相互作用和趋化作用集聚于组织中,然后通过多种细胞因子的作用而活化,并引起促炎症效应和组织的破坏。目前应用最多的HED诊断标准仍然是:符合Chusid于1975年制定的HES的标准,但仅有皮肤损害而无明显内脏损害。系统应用糖皮质激素是治疗HED的首选药物。对于累及心、肺、肝和肾等脏器者要按照HES治疗方案对其尽早治疗。     

特发性嗜酸性粒细胞增高综合征2例并文献回顾

目的了解特发性嗜酸性粒细胞增高综合征(idiopathic hypereosinophilic syndrome,IHES)的临床、病理特点,分析其诊断与治疗方案,提高诊治水平。方法以2例IHES患者为研究对象,结合文献对其临床表现、组织病理、诊断以及治疗方法进行综合分析。结果 2例患者外周血、骨髓、皮损组织病理中均见大量嗜酸性粒细胞,以成熟型为主;诊断为IHES;经泼尼松、α干扰素等治疗有效。结论 IHES缺乏特异性,需排除各种原发及继发因素方可诊断,血象和骨髓检查对鉴别诊断有重要价值;早期诊断、治疗非常重要,糖皮质激素、α干扰素等药物可有效控制病情。夏