类天疱疮和天疱疮患者肿瘤坏死因子α及可溶性肿瘤坏死因子Ⅰ型受体的测定

目的探讨肿瘤坏死因子α（TNFα）则及其可溶性Ⅰ型受体（sTNF-RI）在类天疱疮（BP）、天疱疮（PV）患者疱液和血清中的变化及其临床意义。方法应用ELISA方法对比研究31例BP、9例PV患者血液和疱液中TNFα和sTNF-RI的含量。并以15例正常人吸疱液和30例正常人血清作对照。结果BP、PV患者活动期癌液和血液中sTNF-RI水平均高于对照,且疱液中浓度更高;BP缓解初期血中sTNF-RI水平高于活动期;BP患者血液和疱液中均未测到或有极少量TNFα,PV患者血液中仅测到低浓度的TNF。而疱液中TNFα浓度明显高于对照。结论细胞因子sTNF-RI和TNFα可能参与了BP和PV的发病,sTNF-RI在BP中可能对机体起保护作用,有望成为病情好转和治疗有效的新的观察指标。     

以非水疱为初发皮疹的大疱性类天疱疮34例

目的 探讨以非水疱为初发皮疹的大疱性类天疱疮患者的临床表现、实验室检查和治疗特点。方法 回顾性分析34例以非水疱为初发皮疹的大疱性类天疱疮患者的临床资料。 结果 34例患者中男女之比1.83 ∶ 1，始发年龄平均为（59.79 ± 15.63）岁。在出现典型的水疱之前，表现为红斑、丘疹、丘疱疹、斑块、风团、结节、多形红斑样皮疹等多形性表现，以同时出现红斑、丘疹/斑块（占35.29%）为多见。结论 在所分析的病例中，近1/3大疱性类天疱疮患者初发表现为多形性，以同时出现红斑、丘疹/斑块多见。     

Th17细胞及其衍生白细胞介素17在类天疱疮病理中的作用

近年来,白细胞介素17（IL-17）已逐渐成为皮肤科的研究热点,如银屑病、类风湿性关节炎等疾病与IL-17的关系日渐明朗,但IL-17与类天疱疮, 如大疱性类天疱疮（bullous pemphigoid,BP）、黏膜类天疱疮（mucous membrane pemphigoid,MMP）的相关研究较少。我们主要针对Th17细胞及其衍生IL-17细胞因子在类天疱疮病理发生中的作用做一概述……     

妊娠性类天疱疮1例

患者女,23岁,孕33周。躯干及四肢出现红色皮疹,伴瘙痒2周。皮肤科情况:躯干及四肢广泛分布轻度水肿性红色斑疹和斑丘疹,双手及双足有较多张力性水疱。右前臂皮损组织病理示:真皮乳头高度水肿,水疱形成,疱液及真皮浅层少量嗜酸粒细胞和淋巴细胞浸润。直接免疫荧光检查示:皮肤基底膜带处C3呈线状沉积。诊断:妊娠性类天疱疮。予小剂量强的松口服9d后好转。     

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。     

儿童大疱性类天疱疮

大疱性类天疱疮是一获得性自身免疫性疾病,常累及老年人,儿童少见。儿童大疱性类天疱疮的发病率无明显的人种、性别区别,年龄跨度较大,儿童期各年龄组均有报道。儿童大疱性类天疱疮的诊断主要依据临床表现、组织病理及直接免疫荧光检查。在病因及临床表现上儿童有别于成人,但在组织病理及直接免疫荧光上与成人大疱性类天疱疮相似。     

天疱疮282例和类天疱疮366例回顾性研究

目的 探讨天疱疮和类天疱疮诊断与治疗特点.方法 对2003-2007年天疱疮和类天疱疮648例患者的临床资料进行同顾性分析.结果 天疱疮平均发病年龄低于类天疱疮(P＜0.001),直接免疫荧光(DIF)确诊的175例天疱疮患者中IgG100%阳性,C3 92.0%阳性;类天疱疮223例患者中C399.1%阳性,IgG 51.12%阳性.两种大疱性疾病的诊断中,①天疱疮患者的临床诊断、病理检查与DIF三者一致率为68.8%.病理检查、DIF两者一致率为80.7%.②类天疱疮患者的临床诊断、病珲检查与DIF三者一致率为62.8%.病理检查、DIF两者一致率为78.1%.糖皮质激素是治疗两种疾病的主要手段,泼尼松(0.5～1.5)mg·kg-1·d-1可控制大部分患者病情.结论 临床和病理无法确诊的大疱疮和类天疱疮病例,DIF可以作为诊断该病的重要方法.在基层医院,临床怀疑天疱疮,DIF可主要检查IgG、C3,怀疑类天疱疮,可主要检查C3.     

Management of Ocular Cicatricial Pemphigoid with Intravenous Immunoglobulin Monotherapy

Purpose: To assess the long-term efficacy and safety of IVIg monotherapy in patients with recalcitrant ocular cicatricial pemphigoid (OCP).

Methods: A chart review of all OCP patients seen at the Massachusetts Eye Research and Surgery Institution (MERSI) between 2005 and 2015 was completed. Stage was graded by using the Foster grading system. IVIg infusion was 2g/kg/cycle administered in 3 consecutive days monthly.

Results: Of 512 OCP patients, 17 patients (34 eyes) treated with IVIg monotherapy were identified. Seven were female and ten were male. The average age at diagnosis was 60.7-year-old. The follow up time ranged from 12 to 140 months. Twenty-six eyes (76.5%) achieved remission. Nine remission eyes received cataract surgeries, and 2 of them had relapse (22.2%). The other 17 eyes did not undergo ocular surgery and remained in remission. IVIg monotherapy showed high efficacy in stage 1 OCP (7/7, 100%). Ocular surgery can be associated with OCP relapse (Table 2).

Conclusions: IVIg monotherapy is an effective and safe therapy in patients with recalcitrant OCP. Ocular surgery can be associated with OCP relapse.     

Pemphigus,pemphigoid, and epidermal upper-cytoplasmic antigens: Changes in expression in cultured human keratinocytes

Summary In an approach of epidermal differentiation, the expression of pemphigus, bullous pemphigoid, and upper-cytoplasmic epidermal antigens was studied in human keratinocytes in culture. The cells were cultured without feeder cells, dermal tissue, or collagen at an acid pH (5.6–5.8) similar to that of the surface of the skin in vivo. Cell suspensions from fresh trypsinized skin and primary, secondary, and tertiary cultures were tested by indirect immunofluorescence for the presence of each antigen using human sera from patients with pemphigus, bullous pemphigoid, and human sera with antibodies against upper-cytoplasmic antigens. Normal sera and cultured human normal fibroblasts and melanoma cells were used as controls. Pemphigus and pemphigoid antigens were found to be expressed, and synthesized by keratinocytes in vitro. The expression to upper-cytoplasmic antigens decreased with time in culture, and they were absent in secondary or tertiary cultures while expressed by 45–65% of cells prepared from fresh skin. Both upper-cytoplasmic and pemphigoid antigens can be used to type subpopulations of human epidermal cells; however, these findings suggest that epidermal differentiation in vitro differs from that which occurs in vivo.

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Abreviations BMZ basement zone - BP bullous pemphigoid - FCS fetal calf serum - MEM minimal essential media - IC inter cellular - IF immunofluorescence - PBS phosphate-buffered saline - PV pemphigus vulgaris - U-CYT upper-cytoplasmic Supported in part by a grant from the Fondation de l'Industrie Pharmaceutique pour la Recherche. Paris, France, by Research Grant CA 13844.07 from the USPHS, and by grant PCM 7911783 from the National Science Foundation, USA Offprint request to: Prof. J. Thivolet (address see above)     

Case Report of a Novel Association between Anti-p200 Pemphigoid and Acquired Haemophilia A

Anti-p200 pemphigoid is an uncommon subepidermal autoimmune bullous disease that, unlike many other autoimmune bullous diseases, has not previously been associated with hematological diseases. The diagnosis of anti-p200 pemphigoid in a patient with congruent clinical features requires the demonstration of subepidermal blistering, with linear deposition of immunoglobulin (Ig) G and/or C3 at the dermoepidermal junction on direct immunofluorescence, and a floor-binding pattern on indirect immunofluorescence. In addition, the detection of antibodies against p200 antigen via immunoblotting is ideal but not readily accessible in many facilities, leading to a potential under-recognition and under-diagnosis of this condition. In this case report, we describe a 53-year-old gentleman with recently diagnosed acquired hemophilia A who developed a concurrent vesiculobullous eruption and was evaluated to have anti-p200 pemphigoid. Both of his conditions were controlled with immunosuppression via prednisolone and cyclophosphamide. While we acknowledge the contemporaneous occurrence of both diseases in this patient may be a mere coincidence, it is important to recognize the possibility of this association given the potential clinical significance. Whether the activity of one disease parallels the other will require further evaluation.