A case of bullous transient acantholytic dermatosis

报告1例大疱性暂时性棘层松解性皮病.患者女,22岁.因全身起水疱伴瘙痒2周就诊.组织病理检查显示表皮内海绵状水疱形成,疱液中有棘层松解细胞.直接免疫荧光检查阴性.符合大疱性暂时性棘层松解性皮病的诊断.     

大疱性疥疮

本文报告1例临床酷似大疱性类天疱疮的大庖性疥疮。男,73岁,在胸、背、臀、四肢、阴茎出现瘙痒性结痂性发疹4个月,四肢反复出现水疱2个月。曾用强的松80mg/d治疗14天,后逐渐减量,6周后停药,但水疱仍存在并反复出现。体检见结痂性皮损、炎性斑块和一些疱壁紧张、部分呈出血性的水疱,很像大疱性类天疱疮,但在皮损的搔抓物中发现了疥螨。大疱活组织检查示表皮下水疱,疱腔及表皮下可见大量嗜酸粒细胞,在皮损边界处的乳头层顶部可见真表皮裂隙,PAS染色示疱壁底部基板完整,     

疱疹样天疱疮1例

患者男,67岁,躯干、四肢多发环形红斑、水疱伴痒1个月。既往左髋关节结核病史1年。皮肤科检查全身多发环形红斑,边缘隆起,红斑边缘出现绿豆大小紧张水疱,疱液澄清,部分水疱融合成环形或线状大疱,尼氏征阴性,部分糜烂、结痂,剧烈瘙痒。皮损组织病理检查示表皮轻度角化过度,棘层肥厚,棘细胞间水肿,偶见网状变性小水疱形成,真皮较多嗜酸性粒细胞移入表皮内,浅层血管周围大量淋巴细胞、嗜酸性粒细胞及中性粒细胞。直接免疫荧光:表皮棘细胞间IgG(+),IgA(-),C3(+)。结合临床表现和组织病理改变,诊断为疱疹样天疱疮。     

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。     

局限于外阴和肛周的慢性家族性良性天疱疮1例

患者女,25岁。外阴及肛周散在分布水疱伴瘙痒和疼痛1年。皮损组织病理检查示:角化过度,表皮增生,基底细胞层上水疱,棘层松解细胞见于表皮全层或表皮的下半部,大部分棘层松解细胞只是轻度分离,犹如坍塌的砖墙,可见角化不良的棘层松解细胞。真皮乳头水肿,浅层血管周围以淋巴细胞为主的浸润。诊断:慢性家族性良性天疱疮。     

大疱性疥疮

报告1例大疱性疥疮。患者男,65岁。3个月前无明显诱因躯干、四肢出现散在红斑、水疱、糜烂、结痂,伴瘙痒。患者1个月前指缝间、阴囊处出现类似红斑、丘疹,瘙痒明显。皮肤科检查:躯干、四肢可见弥漫性红斑,部分红斑上可见紧张性水疱,疱壁紧张,疱液清亮,部分水疱破裂后糜烂,可见红色糜烂面,部分表面结痂;双手指缝间、阴囊散在红斑,苔藓样变,可见抓痕。皮损组织病理检查:(左股内侧皮损)角化不全,浆液渗出,可见表皮下疱,疱内可见浆液,大量嗜酸性粒细胞及红细胞,真皮浅层可见嗜酸性粒细胞及中性粒细胞浸润;直接免疫荧光:皮肤全层未见特异性荧光,间接免疫荧光:真皮与表皮交界处(-);镜检发现疥螨。诊断:大疱性疥疮。     

小剂量利妥昔单抗联合糖皮质激素治疗儿童落叶型天疱疮一例疗效观察

患儿男,9岁,头皮红斑、糜烂伴瘙痒1个月,泛发全身1周。皮损组织病理：表皮内颗粒层下方水疱形成,疱内见大量棘层松解细胞,真皮浅层小血管周围淋巴细胞及嗜酸性粒细胞浸润。直接免疫荧光检查示表皮细胞间IgG、补体C3呈网状沉积,IgM、IgA阴性。酶联免疫吸附实验检测血清抗Dsg1抗体阳性（157.00 U/ml）。诊断：落...     

复发性线状棘层松解皮病1例国内首报

报告1例复发性线状棘层松解皮病。患者女,24岁。右大腿内侧反复起红斑、水疱,伴轻度瘙痒20余年。皮损沿Blaschko线分布。皮损组织病理学检查示:表皮增生,棘层肥厚,基底细胞层上裂隙形成,部分表皮裂隙中可见不同层面的单个或倒塌砖墙样棘层松解细胞,真皮乳头轻度水肿,浅层血管周围有较致密的以淋巴细胞为主的炎性细胞浸润。     

落叶性天疱疮和银屑病并发

作者报导一34岁朝鲜妇女,躯干部有瘙痒性斑疹。就诊前两周发现腰部皮损,逐渐发展至腹部,一周后,双上臂出现白色鳞屑性丘疹。查体:腰、腹、四肢有稀疏的蚕豆大小的红斑性湿疹样斑疹,几个水疱和白色鳞屑性丘疹,水疱的尼氏征阳性,白色鳞屑性丘疹有点状出血现象。病理:腰部湿疹样斑疹可见表皮内上部大疱,内含一些棘层松解细胞,真皮血管周围淋巴样细胞浸润。鳞屑性丘疹和同形反应处皮损则显示角化不全,颗粒层缺乏,粒细胞外渗至表皮,也可见到真皮乳头伸长和水肿,真皮血管扩张和淋巴样细胞浸润。直接免疫荧光检查,湿疹样斑疹的周围皮肤表     

疱疹样天疱疮1例

患者，男，71岁。主诉：全身红斑水疱伴瘙痒2年，反复发作。现病史：患者2年前开始躯干、四肢陆续出现散在红斑、水疱，疱易破溃呈糜烂或结痂，瘙痒剧烈。中药治疗效果不佳，皮疹逐渐增多，来我科就诊。经病理检查诊断为红斑型天疱疮，给予强的松60mg qd，雷公藤多甙20mg tid口服，皮疹消退后强的松开始减量。减量期间皮损曾多次反复，近几次复发时躯干、四肢出现多数环形红斑，边缘有密集排列的小水疱，取腹部小水疱做病理检查示：棘层中部水疱，疱内可见多数中性和少量嗜酸粒细胞浸润，真皮浅中层血管周围有淋巴细胞、组织细胞及嗜酸粒细胞浸润（图1）。直接免疫荧光检查（DIF）未发现表皮细胞问IgG、IgA、     

药物诱发的大疱性类天疱疮一例

患者,女,20岁。全身皮肤红斑水疱伴瘙痒1个月。1个月前患者因口服“氨酚黄那敏颗粒”后全身出现红斑水疱。皮肤专科查体：上唇、颈部、胸背、腋下、外阴及双大腿内侧见红斑,其上多发黄豆至蚕豆大小的水疱、大疱,疱壁紧张,部分水疱破溃,上附淡黄色痂,尼氏征(-),部分区域可见虹膜样改变,口腔黏膜、舌部受累,可见散在糜烂、水疱。实验室检查、组织病理、直接免疫荧光结合盐裂试验,诊断为药物诱发的大疱性类天疱疮。经甲泼尼龙等治疗1个月后痊愈停药。     

A case of trophoneurotic bullae

A 57-year-old woman who had sensorimotor polyneuropathy with dysgammaglobulinemia showed pea-sized tense blisters on the fingers. Histopathological features were intraepidermal multilocular bullae and degeneration of capillaries of the papillary dermis with decreased nerve components of the dermis. Clinicopathological findings indicate this case to be trophoneurotic bullae.     

Optical coherence tomography imaging of bullous diseases

Background Optical coherence tomography (OCT) is a non‐invasive optical imaging technique with a micrometer resolution that may potentially offer real‐time bedside imaging of sufficient detail to allow for morphological discrimination between different types of bullae. Objective To explore the potential of OCT in bullous skin disorders by looking at a set of patients with skin blisters of known origin and study the OCT images for possible hallmarks of the blistering level. Materials and methods OCT provides cross‐sectional, tomographic images of the skin. A consecutive series of patients were recruited and their lesions imaged by OCT: 3 patients with bullous pemphigoid (BP), 1 patient with extensive bullae following burns, 1 patient with pemphigus, 1 patient with subcorneal pustular dermatosis, and a patient with Dariers disease. The latter two were included due to similarity to pemphigus with respect to the level of defect cell adhesion. Results In OCT images, BP bullae are easily depicted as dark, ovoid to round well‐demarquated areas, and BP bulla morphology is clearly different from the burn blisters and the pemphigus‐like disease with respect to the blistering level. Discussion Differentiation of epidermal and subepidermal blisters is demonstrated using OCT. The variation within pemphigoid lesions and pemphigus‐like diseases is however too subtle to allow for differential diagnosis; this may be ascribed to limited resolution. Enhanced resolution of OCT may overcome this obstacle.     

新生儿线状IgA大疱性皮病

患儿男, 生后10 d, 因皮肤红斑、水疱6 d就诊入院。皮肤科检查:全身皮肤散在或融合分布红斑, 在正常皮肤或红斑基础上可见大小不等的紧张性水疱, 部分水疱破溃、糜烂;口腔黏膜可见血疱、红色糜烂面。组织病理检查示表皮下水疱, 疱内可见中性粒细胞及少量嗜酸性粒细胞。直接免疫荧光检查显示:沿基底膜带有均质型线状IgA和颗粒状C3沉积, IgG阴性。诊断:新生儿线状IgA大疱性皮病。给予营养支持、防感染等综合治疗后, 皮肤红斑、水疱消退, 黏膜损害减轻出院。患儿出院后16个月电话随访, 一般情况良好, 皮肤黏膜皮损消退、愈合, 无新发皮疹, 生长发育正常。     

获得性大疱性表皮松解症

报告1例获得性大疱性表皮松解症.患者男,56岁.全身摩擦部位皮肤经摩擦后起大疱2年余.2年多以来,躯体受摩擦部位易擦伤、起大疱和结痂,无疼痛和瘙痒,病情反复,且伴有白色丘疹.体格检查可见躯干和四肢有大片红斑、糜烂、水疱和结痂,尤以易摩擦部位为重,双手和耳郭可见白色丘疹,甲变形、断裂.皮损组织病理检查示表皮下大疱和粟丘疹,直接免疫荧光检查在基膜下可见IgG呈线状沉积.根据典型的临床表现、组织病理改变和免疫荧光的检查结果诊断为获得性大疱性表皮松解症.给予糖皮质激素口服治疗.     

大疱性类天疱疮合并原发性腹膜后弥漫大B细胞淋巴瘤1例

患者男,77岁。躯干、四肢反复红斑、水疱1年余,加重10d,确诊为大疱性类天疱疮。患者发病4年后体检时发现腹膜后肿物,外科手术切除肿物后组织病理显示淋巴样细胞弥漫性浸润,免疫组化示CD20(+),PAX-5(+),Bcl-6(灶+),MUM-1(+),Ki-67(90%+)。诊断:原发性腹膜后弥漫大B细胞淋巴瘤。     

儿童获得性大疱性表皮松解症一例

报道1例儿童获得性大疱性表皮松解症.患者女,12岁,因全身反复水疱、大疱和糜烂面3个月入院.患者弱智,父母非近亲结婚,无家族史.入院时表现全身大片糜烂面,包括手足、膝关节周围和股臀部,同时躯干、四肢正常或水肿性红斑基础上紧张性水疱、大疱.治疗过程中突然发生躯干、四肢大量紧张性水疱.组织病理提示,表皮下水疱,免疫荧光结果见表皮基底膜带IgG和C3线状沉积,盐裂后沉积限于真皮侧.ELISA检测BP180和BP230阴性.采用大剂量糖皮质激素联合四环素口服取得满意疗效.

Abstract：

A case of epidermolysis bullosa acquisita (EBA) in childhood is reported. A 12-year-old girl was hospitalized for a 3-month history of recurrent blisters, bullae and erosions on the trunk and limbs. The girl had mental retardation but no family history of similar disorders. The marriage between her parents was not consanguineous. Physical examination on admission revealed large erosions with moderate oozing on the hands and feet, around the knees and on the buttock and thighs. There were scattered tense blisters and bullae arising in normal skin or edematous erythema on the trunk and limbs. During the treatment course the patient suddenly developed a number of tense blisters over the whole integument on the trunk and limbs. Skin biopsy showed subepidermal bullae with moderate perivascular infiltration of neutrophils and eosinophils. Direct immunofluorescence (DIF) revealed linear IgG and C3 deposition along the basal membrane zone, which was on the dermal side of salt-split skin on indirect immunofluorescence (IIF). ELISA detected no serum antiBP180 or -BP230 antibodies in the patient. A diagnosis of EBA was made. The patient was successfully controlled by intravenous steroids combined with oral tetracycline.     

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。     

Pretibial epidermolysis bullosa and hypothyroidism

BACKGROUND: We report a case of primary non-autoimmune hypothyroidism causing pretibial epidermolysis bullosa. CASE REPORT: A 70-year-old man with primary non-autoimmune hypothyroidism developed blisters of different ages on the lateral aspect of both legs. Pathology reported blisters with subepidermal cleavage. Direct immunofluorescence was negative. Electron microscope examination showed a variable cleavage level and diffuse infiltration of a granulous and amorphous microfibrillar substance. After hormone replacement therapy, euthyroidism was associated with a reduction in the number of bullae and finally complete remission. After 12 months follow-up, the patient has not experienced recurrence. DISCUSSION: Recurrence-free clinical improvement after hormone replacement therapy suggests the diagnosis of hypothyroidism pretibial epidermolysis bullosae. Mochizuki et al. described a similar case which rapidly regressed after hormone therapy but where the electron microscope showed a different cleavage level. These bullae appear to result from a mechanical mechanism due to their localization in areas exposed to friction and also to the presence of bullae of different ages. This hypothesis is confirmed by the presence of a variable level of cleavage and a substance dense to electrons at electron microscopy as well as by the skin weakness. Our case confirms the reality of hypothyroidism pretibial epidermolysis bullosa. Thyroid hormones should be assayed in patients presenting pretibial bullae.     

发生于特瑞普利单抗长期治疗后的大疱性类天疱疮二例

例1女,63岁,全身皮疹2个月伴瘙痒,表现为红斑基础上水疱大疱。2年前因直肠肛管恶性黑素瘤行肠周淋巴结清扫术,静脉注射特瑞普利单抗预防性治疗1年,停药后2周出现全身皮疹。上肢红斑处皮损直接免疫荧光示,IgG沿基底膜带沉积;血清盐裂间接免疫荧光,IgG沿表皮侧线状沉积。血清酶联免疫吸附试验示,BP180 > 200 U/...