大疱性色素性荨麻疹1例

目的　介绍 1例大疱性色素性荨麻疹的临床资料并探讨该病的最新研究进展。方法　取臀部新发皮损做皮肤组织病理和Giemsa染色检查真皮内肥大细胞。结果　表皮大致正常 ,表皮下水疱形成 ,真皮内有大量大小一致的、圆形、胞浆淡染的、单一核细胞 ,Giemsa染色示胞浆内有紫红色颗粒 ,证明是肥大细胞。结论　大疱性色素性荨麻疹是肥大细胞增生症的一个特殊类型。     

大疱性色素性荨麻疹1例

患儿男，9个月，因全身皮肤起风团伴瘙痒6个月，出现水疱3个月于2003年3月21日收住院，患儿6个月前无明显诱因全身皮肤起红斑、风团，同时伴瘙痒，皮疹有时会自行消失，遗留色素沉着斑。当地医院诊断为“荨麻疹”，予口服药物治疗（具体药物不详），效果欠佳，风团样皮疹反复出现。其母述3个月前在色素沉着斑处搔抓、摩擦数分钟后可出现风团，甚至水疱和大疱（图1），水疱主要波及颈部、腋窝、腹股沟等皱褶处及头皮、背部。水疱、风团10余天后可消退，遗留色素沉着斑。患儿系足月顺产，出生时阿氏评分10分，母乳喂养，父母非近亲结婚，家族中无类似患者。     

大疱性色素性荨麻疹1例

患儿女,1岁,躯干、四肢反复起斑丘疹、水疱1年伴痒。全身可见褐色斑丘疹,部分斑丘疹上见水疱,摩擦斑丘疹后局部出现风团样损害,即Darier征(+)。皮损组织病理示:表皮下大疱形成,真皮浅层可见大量肥大细胞浸润,Gimsa染色阳性,直接免疫荧光阴性。     

大疱性色素性荨麻疹

报告1例大疱性色素性荨麻疹.患儿男,19个月.16个月时患儿全身反复出现水疱,尼氏征阴性,2个月来出现风团伴瘙痒,Darier 征阳性.皮损组织病理和甲苯胺蓝染色证实为大疱性色素性荨麻疹.给予酮替芬治疗,稍有好转后出院.     

大疱性色素性荨麻疹一例

患儿,女,1岁4个月。躯干部反复出现红斑、水疱伴瘙痒6个月。患儿6个月前躯干部出现红斑、水疱,外用炉甘石洗剂后瘙痒减轻,7~10天水疱破溃愈合后留下色素减退斑。以后病情反复发作,在当地县医院治疗（具体不详）,症状改善,停药后3天复发,当地医院考虑“天疱疮”转诊至我院。患儿系第一胎,足月顺产,家族无类似病史,接种疫苗规律。体格检查：一般情况良好,发育正常。     

大疱性色素性荨麻疹

报告1例大疱性色素性荨麻疹。患儿女,11个月,3个月时患儿躯干、头部反复出现水疱,尼氏征阴性,同时伴有剧烈瘙痒,Darier征阳性。皮损组织病理检查显示表皮下水疱,大量炎性细胞浸润,Giemsa染色阳性。先后给予抗组胺药和糖皮质激素治疗,效果不明显,现口服色甘酸二钠,病情稳定。     

成人色素性荨麻疹1例

色素性荨麻疹（Urticaria pigmentosa,UP）属于皮肤肥大细胞增生症的一种,病因不明,其发病率为1：8000～1：1000之间,男女发病率相当。大多数患者为儿童,成人发病不常见。本文报道1例典型的成人色素性荨麻疹。     

色素性荨麻疹1例

患者女，18岁，全身起疹17例，对其组织切片进行吉姆莎 染色，可见肥大细胞，结合临床符合色素性荨麻疹诊断。     

大疱性荨麻疹1例

患者女,16岁。因全身反复出现风团、水疱2年,再次发作3h,于2004年5月12日来我科就诊。患者平时劳累后皮肤上易出现红斑、风团,24h内可消退。有时部分风团表面起水疱,米粒至黄豆大,持续约1周方消退,消退后局部遗留色素沉着斑。     

大疱性肥大细胞增生症1例

患儿女,1岁6个月。头面部及躯干反复出现水疱、大疱1年余,发生前头面部及躯干潮红伴风团样皮损,后出现水疱、大疱,尼氏征(-),伴瘙痒,darier征(+)。腰部皮损组织病理示:表皮下水疱,大量炎性细胞浸润,Giemsa染色(+)。诊断:大疱性肥大细胞增生症。     

大疱性肥大细胞增生病1例

患儿男,4个月。头皮、躯干皮肤水疱、大疱3天。系统检查无异常。皮损组织病理:表皮大致正常,表皮下水疱形成。Giemsa染色示真皮内胞质有大量紫红色异染颗粒的肥大细胞。结合临床表现诊断:大疱性肥大细胞增生病。     

大疱性肥大细胞增生症1例

患儿女,13个月。躯干反复出现水疱和大疱3月、风团伴瘙痒1月。皮损组织病理示:表皮下大疱形成,真皮中上部弥漫性疑似肥大细胞浸润。甲苯胺蓝染色示:细胞质内有紫红色异染性颗粒。诊断:大疱性肥大细胞增生症。     

婴儿大疱性类天疱疮1例

婴儿男,6月大时出现全身红斑、水疱和大疱,皮损组织病理可见真皮内嗜酸性细胞浸润,直接及间接免疫荧光显示:IgG基底膜带线状沉积。诊断:婴儿大疱性类天疱疮。糖皮质激素治疗有效。1岁以内婴儿大疱性类天疱疮国内报道少见。     

大疱性系统性红斑狼疮1例

报告1例大疱性系统性红斑狼疮。患者女,22岁。2009年5月逐渐出现头痛、关节痛、牙龈出血、呕血、周身瘀点、瘀斑、血小板减少。此次因口腔反复溃疡4月余,周身红斑水疱1月余,视物不清1周就诊。临床表现为红斑、水疱、口腔溃疡。实验室检查符合SLE诊断。背部水疱处组织病理检查示:表皮下水疱形成,内含中性粒细胞、淋巴细胞及纤维素网,基底细胞液化变性。毛囊周围较多淋巴细胞浸润。直接免疫荧光显示IgG,C3基底膜带线状荧光沉积。     

大疱性系统性红斑狼疮1例

患者女,38岁。确诊为系统性红斑狼疮1年。近3周来,全身出现粟粒大紧张或松弛性水疱,尼氏征(-),皮损组织病理示:表皮下疱形成,直接免疫荧光示:IgA,IgG,IgM和C3线状沉积于基底膜带。诊断:大疱性系统性红斑狼疮。予泼尼松和沙利度胺治疗2周后,症状已被控制。     

Mastocytosis: recent advances in defining the disease

Mastocytosis is a rare disease characterized by a primary pathological increase in mast cells in different tissues, which may present in a variety of clinical patterns. Major advances have been made in recent years in the understanding of the pathogenesis of mastocytosis. This review is aimed at familiarizing dermatologists with these recent findings, and at exploring their possible implications for the diagnosis and treatment of the condition. The heterogeneous clinical presentation of mastocytosis is detailed with respect to the type of skin lesions, age at onset, family history, organ systems involved, associated haematological disorders and prognosis. Recent genetic findings also indicate different pathogenetic forms of mastocytosis, as adult patients and those with associated haematological diseases usually express activating mutations of the stem cell factor receptor c-kit, whereas most cases of childhood-onset and familial mastocytosis seem to lack these mutations. Despite the presence of c-kit mutations, patients with cutaneous lesions generally have a good prognosis, even when there is involvement of other organs. Some patients, particularly those with childhood-onset disease, experience spontaneous remission, mostly by puberty. c-kit mutations do not explain the initial cause of mastocytosis, and their prognostic significance is as yet unclarified, as is the pathogenesis in patients without the mutations. Furthermore, these novel findings have as yet not resulted in a more effective treatment of the cause of the disease, so that counselling, prevention of exposure to mast cell secretory stimuli, and symptomatic treatment remain the mainstays of current patient management.     

大疱性类天疱疮合并胰头癌1例

患者男,86岁。躯干及四肢散在分布红斑和水疱,水疱呈半球形,疱壁紧张,较厚,尼氏征阴性。皮损组织病理检查示:表皮下水疱形成,疱内可见纤维素及嗜酸性粒细胞,真皮内散在嗜酸性粒细胞及中性粒细胞浸润。15个月后检查腹部CT示:胰头增大,胆总管扩张明显,内有占位影。诊断:大疱性类天疱疮;胰头癌。     

The efficacy of omalizumab in Cutaneous Mastocytosis: A case series

Background: Mastocytosis describes a heterogeneous group of disorders arising from a clonal proliferation of mast cells. Given the lack of curative treatments for the cutaneous form, there is a significant need for superior therapies. Omalizumab is a recombinant DNA‐derived humanized IgG monoclonal antibody that selectively binds to human immunoglobulin E (IgE). It represents a potential treatment for the management of cutaneous mastocytosis, which currently has no standard treatment. Methods: Two patients were treated with subcutaneous omalizumab 300 mg every 4 weeks. Discussion: Patient 1 experienced 50% reduction in cutaneous infiltration and moderate improvement in pruritus. Patient 2 underwent 90% complete clearance of cutaneous lesions and reported full resolution of pruritus. The median duration of treatment was 24 weeks and time to response was 8 weeks. No significant changes in tryptase levels were observed. Both patients experienced injection site reactions. Conclusion: We provide evidence from two cases supporting the efficacy of IgE‐mediated therapy in the treatment of cutaneous mastocytosis. Even at a higher‐than‐standard dose (300 mg vs. 150 mg), the drug was well‐tolerated. As we await the results of pivotal clinical trials, omalizumab appears to be a promising treatment option in patients with cutaneous mastocytosis unresponsive to traditional therapies.