妊娠疱疹

报告1例妊娠疱疹。患者女,24岁。妊娠22^＋周。在妊娠8^＋周时全身出现红斑、水疱,伴瘙痒,持续2个月后症状加重。组织病理检查示表皮下水疱,直接免疫荧光检查示基膜带IgG、C3呈线状沉积。     

PD-1抑制剂治疗原发性肝癌出现大疱性类天疱疮一例

47岁男性患者,躯干、四肢皮损伴瘙痒2个月。患者1年前因原发性肝癌行信迪利单抗免疫治疗,2个月前躯干、四肢出现红斑、水疱。右下腹红斑组织病理检查示表皮下水疱,左大腿红斑直接免疫荧光示Ig G沿基膜带沉积。血清BP180抗体> 200 RU/ml。诊断：大疱性类天疱疮。予甲泼尼龙40mg/d静脉滴注,辅以卤米松乳膏外用,经治疗红斑变暗,水疱干涸、结痂。     

结节型类天疱疮1例

患者女性，55岁，因全身反复起红斑，丘疹，结节1年入院，体检。头颈，躯干，四肢红斑，丘疹，色素沉着斑，四肢散在多数绿豆至花生米大小结节，多数结节顶端见糜烂或结痂，未见水疱，间接免疫荧光抗类天疱疮抗体阳性，直接免疫荧光示IgG沿基底膜带呈线状沉积，组织病理示表皮下裂隙性水疱，诊断为结节型类天疱疹，予小剂量皮质类固醇合用氨苯砜治疗后取得较满意疗效。     

疱疹样天疱疮3例并文献复习

报道疱疹样天疱疮3例,其中男2例,女1例,平均年龄67岁;主要表现为全身皮肤红斑、水疱。组织病理检查示表皮内水疱,真皮浅中层嗜酸粒细胞浸润,直接免疫荧光表皮细胞间IgG沉积。 1例患者接受了间接免疫荧光检查,抗天疱疮抗体滴度为1:80,氨苯砜和糖皮质激素治疗有效。     

青年女性接触精油后出现大疱性类天疱疮1例

患者女,34岁。全身皮疹伴瘙痒1个月。皮疹在外用精油1周后出现,初始发生在接触部位,继而泛发全身,皮损以浮肿性红斑及水疱为主。皮肤组织病理提示表皮下水疱,真皮上层及水疱内可见嗜酸性粒细胞和中性粒细胞炎性浸润。直接免疫荧光:基底膜带线状连续的IgG,C_3沉积。诊断:大疱性类天疱疮。予糖皮质激素联合丙种球蛋白治疗的效果满意。     

儿童获得性大疱性表皮松解症二例北大核心CSCD

例1 男,7岁,因全身红斑水疱5年、加重1周就诊。患儿5年前无明显诱因口周、颈部、股部出现红斑、水疱,后水疱逐渐蔓延至全身,于当地医院诊断为线状IgA大疱性皮病,病理检查示表皮下水疱,疱液及真皮可见大量中性粒细胞、淋巴细胞浸润,直接免疫荧光示IgG、C3基底膜带线性沉积,IgA、IgM均阴性,予甲泼尼龙（16 mg/d）、环孢素（50 mg每日2次）口服,病情控制,药物逐渐减量,1年后病情复发加重,     

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...     

红斑型天疱疮误诊1例

 报告红斑型天疱疮误诊1例。患者女,42岁,全身红斑、鳞屑1年。皮肤科检查：面部对称性水肿性红斑,上覆黄白色细痂屑;躯干、四肢见环形浸润性暗红斑,边缘稍隆起,中央消退见褐色色沉,边界清楚,腹部红斑基础上见3个黄豆大小的水疱,疱液清亮,疱壁松弛,尼氏征可疑阳性。天疱疮抗体Dsg1、Dsg3均阳性。面部皮损组织病理结果示：角化不全,角质层及棘层上部裂隙,可见棘突松解现象。直接免疫荧光：棘层上方IgG网状沉积。诊断：红斑型天疱疮。予泼尼松片、羟氯喹联合甲氨蝶呤片治疗后,面部红斑减轻,躯干皮损消退后出院,随访1年皮疹无复发。     

银屑病并发大疱性类天疱疮

例1.男,32岁。全身鳞屑性红斑17年,臀部及腰部水疱2个月。皮肤科检查：躯干及四肢可见散在红斑,表面少许鳞屑,腰腹部可见成簇紧张性水疱,疱液清亮,尼氏征阴性,无口腔及会阴糜烂。取水疱行组织病理检查,结果示表皮下水疱,直接免疫荧光示IgG、Ig M和C3沿基膜呈线状沉积。诊断：寻常性银屑病;大疱性类天疱疮。予克拉霉素口服及哈西奈德乳膏外用病情好转。例2.男,72岁。全身鳞屑性红色斑块32年,散在水疱7 d。皮肤科检查：躯干及四肢多发鳞屑性红色斑块,上臂及腹部正常皮肤及红色斑块边缘散在水疱,水疱松弛,部分水疱表面破裂形成糜烂,无口腔及会阴糜烂,无疼痛,尼氏征阴性。取水疱行病理检查,示表皮下水疱,直接免疫荧光C3沿基膜呈线状沉积,IgG、IgM及IgA（-）。诊断：寻常性银屑病;大疱性类天疱疮。予地塞米松10 mg,静脉滴注,每日1次,卤米松三氯生乳膏外用,病情好转。     

盘状红斑狼疮和疱疹样皮炎共存

本文报告1例48岁男性患者,1978年在暴光部位发现皮疹.活检符合盘状红斑狼疮(DLE).免疫荧光显示在真皮与表皮交界处有颗粒性IgG及C_3呈线形沉积,符合DLE.1980年仍有LE皮疹,但此时在躯干上部、前臂和小腿伸侧出现水疱大疱损害.直接免疫荧光示:沿真皮表皮交界区域有很强的IgA颗粒沉积及稀疏的IgG和IgM沉积.患者HLAB_3和DR_3抗原阳性.这些发现符合疱疹样     

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.     

无水疱性类天疱疮二例并文献复习

报道2例无水疱性类天疱疮并复习以往相关文献。例1，男，72岁，因全身皮肤红斑、丘疹伴瘙痒20天就诊，查体未见水疱。实验室检查血嗜酸性粒细胞升高，直接免疫荧光示基底膜带线状C3沉积，间接免疫荧光示抗基底膜带抗体阳性，抗BP230抗体、抗BP180抗体阳性。例2，女，79岁。因全身皮肤散在分布丘疹伴严重瘙痒20天就诊，查体见躯干四肢皮肤抓痕明显，未见水疱。血清中抗BP180抗体阳性。2例均诊断：无水疱性类天疱疮。予以小剂量糖皮质激素治疗后，控制病情，目前在随访中。     

增殖型类天疱疮一例

【摘要】 患者女,89岁,因腹股沟、外阴、臀间沟皮疹10个月、水疱3周就诊。皮肤科检查：腹股沟、外阴、臀间沟红白色增生性斑块,斑块部位散在绿豆至豌豆大小的糜烂面及水疱,右腋下及右小腿正常皮肤上散在数个类似水疱,部分破溃结痂。臀间沟皮损组织病理检查：棘层增生肥厚,无棘刺松解,部分区域可见表皮下裂隙和水疱,局灶性真皮浅层水肿,伴嗜酸性粒细胞浸润,真皮浅层血管周围可见淋巴细胞为主的浸润。直接免疫荧光：IgG、C3基底膜带线状沉积,IgM真皮簇状小体阳性,IgA阴性。盐裂皮肤间接免疫荧光：IgG、C3沉积在表皮侧。酶联免疫吸附试验检测血清抗体：BP180抗体26.92 U/ml、BP230抗体68.17 U/ml,桥粒芯蛋白1抗体、桥粒芯蛋白3抗体正常。诊断：增殖型类天疱疮。予口服甲泼尼龙,联合外用卤米松软膏及0.03%他克莫司软膏,皮疹逐渐消退。     

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。     

病情与外周血嗜酸粒细胞计数平行的大疱性类天疱疮一例

报告1例病情与外周血嗜酸粒细胞计数相平行的大疱性类天疱疮.患者男,65岁.全身泛发红斑、水疱1个月,加重15天.皮肤科检查:皮损泛发头面颈、躯干、四肢及会阴部,基本损害为正常皮肤或红斑基础上出现大小不等的水疱,疱液为草黄色,散在红色糜烂面,尼氏征阴性,部分皮损表面敷以灰褐色痂片,以胸背部、四肢尤为严重.组织病理、直接免疫荧光、间接免疫荧光及盐裂皮肤直接免疫荧光检查结果符合大疱性类天疱疮.入院后依据病情变化,先后给予3次糖皮质激素冲击治疗,治疗过程中,定期监测血常规,提示外周血嗜酸粒细胞计数随病情波动.     

利格列汀相关的BP230型大疱性类天疱疮一例国内首报

患者女,66岁,因头皮、颈部水疱1个月就诊。既往有2型糖尿病病史6年,于起疹前6个月开始口服二肽基肽酶4抑制剂利格列汀5 mg每日1次。皮肤科检查：头皮散在绿豆至花生米大小水疱,部分水疱破溃,可见渗出、结痂;左侧颈部一鸽蛋大小大疱及2个绿豆大小水疱,疱壁紧张,疱液清亮,基底无明显红斑,尼氏征阴性。酶联免疫吸附试验示抗B...     

眼周局限性大疱性类天疱疮一例

患者,女,12岁.眼周反复红斑、水疱4个月,伴瘙痒.皮损组织病理示:表皮下水疱,真皮浅层淋巴细胞、嗜酸粒细胞、中性粒细胞等炎性细胞浸润.直接免疫荧光(DIF):表皮基底膜IgG、C3阳性线状沉积.ELISA检测:血清抗BP230抗体2.6 U/mL,抗BP180抗体阴性.诊断:局限性大疱性类天疱疮.予以甲泼尼龙、氨苯砜...     

Relapse‐associated autoantibodies to BP180 in a patient with anti‐p200 pemphigoid

Anti‐p200 pemphigoid and bullous pemphigoid (BP) are autoimmune subepidermal blistering diseases characterized by autoantibodies to a 200‐kDa dermal antigen (p200) and two hemidesmosomal proteins (BP180 and BP230), respectively. We report a 70‐year‐old man with haemorrhagic blisters, widespread crusted erosions, and the immunopathological characteristics of anti‐p200 pemphigoid. Treatment with doxycycline, topical corticosteroids and immunoadsorption led to rapid clinical remission. However, 19 weeks later, a relapse occurred with generalized itchy urticarial erythema and tense blisters. At this time, both strong dermal and epidermal IgG staining was detected by indirect immunofluorescence microscopy on salt‐split skin, and autoantibodies against both p200 and the 16th noncollagenous (NC16A) domain of BP180 were found. Interestingly, the relapse was associated not only with the detection of autoantibodies to a second autoantigen (BP180), but also with an altered clinical phenotype. This case was a unique occasion to directly monitor the emergence of intermolecular epitope spreading during the course of an autoimmune bullous disorder.     

Penicillin-induced anti-p200 pemphigoid: an unusual morphology

We report here a case of a 52-year-old woman with erythema gyratum repens-like lesions appearing during anti-p200 pemphigoid, probably induced by oral penicillin. The diagnosis of anti-p200 pemphigoid was made by the presence of in vivo bound and circulating IgG anti-basement membrane zone auto-antibody reactive with the dermal side of salt-split skin and with 200 kDa protein in dermal extract on Western immunoblot. Laser scanning confocal microscopic study disclosed the localization of IgG at the lamina lucida-lamina densa border. Skin lesions responded poorly to high dose of prednisone and the combination of prednisone and dapsone. When methotrexate was added, skin lesions healed within 3 weeks. To our knowledge, erythema gyratum repens-like lesions have not been described previously in this disorder. Thus, we have expanded the clinical morphological spectrum of patients with anti-p200 pemphigoid and first described a patient whose disorder was probably drug-induced.     

无水疱性类天疱疮一例

患者，男，70岁。全身泛发多形性红斑、丘疹伴瘙痒3个月，未见水疱。皮肤病理示真皮浅层血管周围轻度淋巴细胞、嗜酸粒细胞浸润。直接免疫荧光示表皮细胞间及基底膜IgG、C3、IgM、IgA阴性。间接免疫荧光示抗基底膜带IgG抗体1:40阳性。正常人皮肤盐裂间接免疫荧光示IgG表皮侧阳性。诊断为无水疱性类天疱疮，给予烟酰胺、四环素、雷公藤多苷口服，配合外用卤米松乳膏治疗，2个月后皮损基本消退。