良性黏膜类天疱疮一例

患者女,83岁.12年前无明显诱因于四肢出现红斑、水疱、糜烂,伴瘙痒,于我院病理诊断为大疱性类天疱疮,予泼尼松30 mg/d口服,效果良好.此后病情反复发作,冬重夏轻,渐累及躯干及腹股沟、腋下等间擦部位.10年前开始自觉眼部发痒、异物感,眨眼渐频繁,自觉睁眼困难,眼球转动稍受限,无眼干、眼痛、眼肌疲劳,无脓性分泌物、视物不清.3年前开始出现口腔内水疱、糜烂,自觉疼痛,影响进食,于我院口腔科给予复方氯己定含漱液、生理氯化钠溶液含漱等治疗稍好转.1年前于我院耳鼻喉科就诊发现食道狭窄,未予治疗.既往患高血压病10余年,药物控制后血压正常.家族中无类似疾病病史.     

妊娠性类天疱疮一例

27岁女性患者,躯干、四肢红斑伴痒4个月余,加重并出现水疱1 d。患者4个多月前(妊娠20~+周)四肢出现红斑、风团伴剧烈瘙痒,后逐渐扩散至躯干。1 d前(妊娠39~+5周)患者自然分娩后红斑加重,四肢出现张力性水疱、大疱,伴痛痒。体格检查:四肢、躯干大片红斑、丘疹,双上肢及股内侧红斑上见张力性水疱、大疱。红斑处组织病理示:表皮下裂隙性疱,真皮浅层血管扩张,血管周围炎性细胞浸润,有较多嗜酸粒细胞。直接免疫荧光示:C3、IgG在基膜带呈线状沉积。诊断:妊娠疱疹。未予治疗,病情逐渐好转,分娩后第20天全身水疱消退,未见新发皮损。     

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...     

黏膜类天疱疮的研究进展

黏膜类天疱疮是一组以黏膜受累为主的慢性、炎症性、自身免疫性上皮下水疱病.最常见的病变为累及任何黏膜部位反复出现的水疱、大疱伴糜烂.皮损愈合后形成的瘢痕是其特异性的临床标志.发病主要与多种抗基底膜带的自身抗原相关,包括BP180、BP230,层黏连蛋白332、整合素α6β4等.诊断需基于典型的临床表现及直接免疫荧光.治疗...     

黏膜类天疱疮的研究进展

黏膜类天疱疮是指一组慢性、异质性、自身免疫性表皮下水疱病,主要累及口腔、眼睛等黏膜,其特点是具有形成瘢痕的风险。患者体内存在抗基底膜带的自身抗体,分别是针对BP180、BP230、层粘连蛋白332、整合素α6β4,和Ⅶ型胶原,其中BP180的羧基端、层粘连蛋白332被认为是主要的自身抗原。诊断主要基于临床表现、组织病理及免疫学检查。治疗上应根据受累部位,疾病严重程度及进展速度,选择合适的治疗方案,为了尽量减少不可逆的损害,多学科合作诊疗是必要的。     

儿童大疱性类天疱疮一例

患儿男，1岁8个月，因全身泛发大疱伴轻微瘙痒2个月于2002年9月29日入院，2个月前无明显诱因全身泛发绿豆至核桃大小的水疱，伴全身轻微瘙痒，水疱破溃后可迅速愈合，不留瘢痕，无发热，糜烂，溃疡，在当地医院治疗，效果欠佳，皮疹逐渐增多。既往健康，足月顺产，家族成员中无类似疾病史，入院体检：体温36.8℃，脉搏 80，呼吸20，体重11kg。     

幼儿大疱性类天疱疮一例

1970年Bean等^[1]首次报告1例免疫荧光(IF)阳性的小儿大疱性类天疱疮(BP),至1978年共报告14例,现介绍所见一例.     

妊娠类天疱疮一例

本文报道1例36岁女性患者，分娩后全身发生红色丘疹18天，水疱11天就诊。常规病理及免疫病理示大疱性类天疱疮样改变，诊断为妊娠类天疱疮。     

黏膜性类天疱疮68例临床回顾分析北大核心CSCD

目的总结1987年8月至2012年10月我科诊治的68例黏膜性类天疱疮的临床特点和治疗经验。方法总结黏膜性类天疱疮患者黏膜皮肤损害、组织病理改变、免疫学等方面的特点,重点分析治疗方案。结果黏膜性类天疱疮68例,累及口腔黏膜67例占98．5％,眼部黏膜23例占33．8％。免疫学检查：16例行直接免疫荧光检查（DIF）,8例阳性;39例行间接免疫荧光检查（IIF）,8例阳性,阳性率20．5％;41例采用ELISA法检测血清中抗BP180抗体,22例阳性,阳性率53．7％。依据皮损分布确定病情严重程度后,予以局部治疗5例;中低剂量糖皮质激素系统治疗（0．4—0．5mg·kg^-1·d^-1,或30mg／d）55例,其中3例同时累及皮肤、口腔黏膜、眼结膜及其他黏膜的患者剂量增加至50mg／d左右才能控制病情。控制剂量起效时间（11．80±5．88）d,控制剂量服药时间0．23～12（3．06±2．84）个月。15例患者基本治愈,其中4例完全治愈。基本治愈患者糖皮质激素剂量减至原剂量50％所需时间为（13．29±5．76）个月,达到基本治愈所需时间为（17．33±7．71）个月。病情控制6个月后,剂量平均减至控制剂量的76．5％,12个月后剂量平均减至控制剂量的58．1％。3例患者在治疗过程中出现口腔念珠菌感染。结论黏膜性类天疱疮的诊断目前主要依据典型的临床和病理表现,尚缺乏灵敏度高的免疫学检查手段。中低剂量糖皮质激素系统治疗,配合局部治疗,常可达满意疗效。     

Ocular mucous membrane pemphigoid

Mucous membrane pemphigoid (MMP) is a systemic disease affecting the mucosal surfaces of the eyes, nose, mouth, upper respiratory, and gastrointestinal tracts. Ocular MMP causes inflammation and scarring of the conjunctival surface of the eye that can lead to blindness if not properly treated. Oral corticosteroids, dapsone, and immunosuppressive agents are used to treat ocular MMP with variable success. The most potent and successful treatment for ocular MMP has been daily oral cyclophosphamide, typically in combination with a limited course of oral prednisone. This regimen has been shown to be effective in controlling the ocular inflammation associated with the disease and halting the progression of scarring of the conjunctiva, the major source of ocular morbidity in this disease. Long-term, drug-free remissions have been reported after 12–24 months of this therapy. This article describes treatment modalities for ocular MMP, efficacies, and drug-related side effects associated with treatment, and provides recommendations for follow-up and monitoring of patients with ocular disease.     

An unusual case of benign mucous membrane pemphigoid.

This case of benign mucous membrane pemphigoid (BMMP) is unusual in that blistering, scarring lesions were confined to the skin for 15 years before mucous membranes were involved. The onset of this disorder at the age of 38 is also unusual. Detailed immunological investigation was performed on this patient but the results in no way clarify the present confusion regarding the immunopathological processes in BMMP related to those operative in bullous pemphigoid.     

Deafness complicating mucous membrane pemphigoid: a case report

A patient with moderately severe mucous membrane pemphigoid is described. The onset of deafness was associated with the appearance of postero-superior retraction pockets in both eardrums. It is postulated that this was due to mucous membrane pemphigoid involving the mucosa of the middle ear. We are not aware of this complication having been described previously.     

Pathogenesis of mucous membrane pemphigoid

Mucous membrane pemphigoid (MMP) is the clinical phenotype of a group of autoimmune blistering diseases characterized by autoantibodies directed against different structural proteins in epidermal basement membranes. The clinical course and prognosis of MMP are affected by the specific autoantigen targeted, the titer and bioactivity profile of corresponding autoantibodies, and the specific mucosal sites of disease activity. Irreversible scarring and loss of function must be prevented by early diagnosis and appropriate interventions.