疱疹样皮炎的IgA及IgA亚型研究

用免疫荧光法对１４例ＤＨ病人的ＩｇＡ及其亚型ＩｇＡ１和ＩｇＡ２进行研究。发现损害周围皮肤的真皮乳头部与沿基底膜带（ＢＭＺ）同时出现ＩｇＡ的颗粒状沉积是ＤＨ的最常见特征。证实部分ＤＨ病人血清中确实存在循环ＩｇＡ抗体。皮肤沉积中只有ＩｇＡ１亚型。分离皮肤间接免疫荧光法的敏感性明显高于传统的间接法。     

伴有谷蛋白敏感性肠病的疱疹样皮炎一例

疱疹样皮炎是一种与谷蛋白敏感性有关的慢性、自身免疫性水疱性疾病,是腹腔疾病的一种皮肤表现。本文报道了一例伴谷蛋白敏感性肠病的疱疹样皮炎患者,严格控制无谷蛋白饮食和氨苯砜治疗后皮损明显好转。     

A Case of Pemphigus Herpetiformis with Only Immunoglobulin G Anti-Desmocollin 3 Antibodies

Pemphigus represents a group of autoimmune blistering diseases caused by autoantibodies against desmogleins (Dsgs), a class of desmosomal cadherins. Recently, several pemphigus patients only with desmocollin (Dsc) 3-specific antibodies have been reported. Here, we report a case of pemphigus herpetiformis (PH), where only anti-Dsc3-specific antibodies but not anti-Dsg antibodies were detected. A 76-year-old woman presented with a 3-year history of blister formation. Physical examination revealed pruritic erythemas with vesicles on the trunk and legs, but no lesions of the oral mucosa. A skin biopsy specimen revealed intraepidermal blister containing neutrophils, eosinophils, and lymphocytes. Direct immunofluorescence (IF) showed immunoglobulin G (IgG) and complement 3 (C3) depositions on the keratinocyte cell surfaces. Indirect IF showed IgG anti-keratinocyte cell surface antibodies. These findings hinted at a diagnosis of pemphigus. However, repeated enzyme-linked immunosorbent assays (ELISAs) for both anti-Dsg1 and 3 antibodies proved to be negative. Immunoblotting of normal human epidermal extracts revealed Dsc antibodies, and recently established ELISAs using human Dsc1-Dsc3 recombinantly expressed in mammalian cells detected anti-Dsc3 antibodies. Based on these clinical, histopathological, and immunological findings, the patient was diagnosed as PH with only anti-Dsc3 antibodies. Treatment with corticosteroid prednisolone and steroid-sparing agent dapsone accomplished complete clinical remission of the patient.     

免疫球蛋白联合小剂量糖皮质激素治愈疱疹样脓疱病1例

患者女,19岁,孕34周,初次妊娠。躯干、四肢皮肤出现脓疱20d。查体：躯干、四肢皮肤浸润性红斑,其上见大量浅表白色脓疱,在红斑边缘聚集成群或排列成环形。皮损组织病理示：局灶性角化不全,角质层下中性粒细胞性脓疱,血管周围淋巴细胞及中性粒细胞浸润。临床结合病理表现符合疱疹样脓疱病诊断。患者对大剂量糖皮质激素反应不佳。给予静滴免疫球蛋白联合小剂量糖皮质激素,皮损痊愈。     

TNF-α拮抗剂治疗复发性疱疹样脓疱病一例

患者,女,31岁。妊娠期反复脓疱加重1个月。患者8年前妊娠期曾全身出现红斑、脓疱,产后皮损自行消失。根据患者既往病史、症状、体征及辅助检查,诊断“疱疹样脓疱病”,入院后予TNF-α拮抗剂皮下注射,辅以复方甘草酸苷、阿奇霉素抗炎及外用药治疗,1周后患者皮疹明显好转出院。     

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。     

疱疹样天疱疮17例分析

目的探讨疱疹样天疱疮的临床特点、治疗及预后。方法对本院1998-2009年收治的17例疱疹样天疱疮住院患者的临床资料作回顾性分析。结果本病组病例临床以泛发性环状红斑、紧张性水疱为特点,尼氏征阳性仅4(23.53%)例,口腔黏膜受累2(11.76%)例,均伴有剧烈瘙痒。皮损组织病理示:表皮棘层内水疱及嗜酸性粒细胞海绵形成,直接免疫荧光示:棘细胞间IgG(或)和C3沉积。8例予氨苯砜100mg/d治疗,3例7天内有效的控制了病情,另5例无效者加用强的松25～50mg/d,10天内也有效的控制了病情;9例予甲基强的松龙20～40mg/d治疗,3天内4例也有效的控制了病情;另5例无效者,分别予激素加量,或加用雷公藤多甙、硫唑嘌呤、环磷酰胺或丙种球蛋白治疗,14天内4例有效的控制病情,其中1例无效者,予血液透析及血液灌流7次,27天内也有效的控制了病情。结论疱疹样疱疮是有特殊的临床表现及病理特点的一类天疱疮,多数病例单用氨苯砜或加用中小剂量糖皮质激素可控制病情。     

疱疹样皮炎的血清学研究进展

疱疹样皮炎是一种与肠病有关的谷胶敏感性皮肤病，皮损表现为瘙痒性的红斑、丘疹、水疱，直接免疫荧光所见的IgA在真皮乳头层颗粒状沉积对疱疹样皮炎诊断有重要价值。尽管病理学检查和直接免疫荧光一直被认为是疱疹样皮炎诊断的金标准，但对于一些症状不典型或取材位置不当无法确诊的病例，血清学检查有着不可替代的作用，血清学检查对于疱疹样皮炎患者的疗效评估和随访均具有重要价值。随着对疱疹样皮炎发病机制的不断认识，新的血清学检测技术也在不断进步，同时通过血清学检查对DH患者血清中多种抗体水平的分析，为疱疹样皮炎发病机制的研究提供了更多线索。     

A case of impetigo herpetiformis associated with intrahepatic cholestasis of pregnancy

We herein reported a 26-year-old primigravida with impetigo herpetiformis and intrahepatic cholestasis of gestation. She was in gestation for 34 weeks. She had a 5-month history of pustules on the trunk and limbs that had exacerbated from 1 month before. The jaundice had also emerged lately with increased levels of serous transaminase, bilirubin and bile acid. The histopathology of the lesion confirmed the diagnosis of impetigo herpetiformis. After cesarean section and administration of glucocorticoid, the patient totally recovered.     

Antiendomysial antibody — useful serological indicator of dermatitis herpetiformis

Summary Antiendomysial antibodies (EmA) of the IgA class are directed against reticulin components of the primate smooth muscle and are markers of gluten-sensitive enteropathy. These antibodies occur in essentially all active cases of celiac disease and in about 70% of dermatitis herpetiformis (DH) patients. IgA deposits in the dermal papillae of the skin are the hallmark of DH and are employed routinely in establishing its diagnosis. The incidence of IgA deposits in skin varies depending upon the site and type of biopsy specimen taken.We studied sera and skin biopsy specimens for EmA and for IgA deposits in the skin from 11 DH patients. EmA were detected in the sera of 10 of the 11 cases. Of these 11 patients, 9 were positive for IgA deposits in their skin, as revealed by direct immunofluorescence (IF). The immune deposits were detected in the normal, and not in the lesional skin. DH cases that were initially negative on biopsy and serum positive for EmA were found to be positive when a repeat biopsy of the normal skin was performed. Thus, serological studies in conjunction with direct IF studies of the normal skin are useful in making a diagnosis of DH.Presented at the Society for Investigative Dermatology Meeting, Washington, DC, May 2, 1986     

Circulating IgA- and IgG-class antigliadin antibodies in dermatitis herpetiformis detected by enzyme-linked immunosorbent assay

Summary A sensitive and technically simple enzymelinked immunosorbent assay (ELISA) was developed to demonstrate circulating IgA- and IgG-class antibodies to gliadin, a component of wheat gluten. Serum samples from 24 patients with dermatitis herpetiformis (DH), 5 with coeliac disease (CD) and 75 normal controls were analysed. Antigliadin antibodies (AGA) of the IgA class were detected in 71% of DH patients, all of the CD patients and 19% of the controls. IgG-AGA was found in over 90% of DH patients and controls and in all of the CD patients. The mean ELISA values of both IgA- and IgG-class AGA were significantly higher in DH patients than in the controls. The occurrence of circulating IgA-class AGA is compatible with the hypothesis that these antibodies can be deposited in the skin, e.g. as immune complexes, or due to cross-reactivity of gliadin and dermal reticulin.Supported by grants from the Foundation for Finnish Chemical Research     

疱疹样脓疱病23例临床与病理分析

为了了解疱疹样脓疱病的临床与病理特点，对23例病人进行了分析。在临床上，损害常为成片状脓湖，3－4天后脓疱干枯，形成黄色薄痂或脱屑，经经过7－10天新脓疱又现，呈周期性发作，病理上，取新脓疱活检，发现角质形成细胞变性，表皮水肿，表皮内有中性粒细胞移入，棘层上部海绵样脓疱形成，真皮血管扩张，血管周围淋巴细胞及中性粒细胞浸润，最后在治疗上作了讨论。     

IgA anti-endomysial antibody detection in the serum of patients with dermatitis herpetiformis following gluten challenge

Summary This study reports the appearance of IgA-class anti-endomysial antibodies in the serum of 8 out of 12 patients with dermatitis herpetiformis who were challenged with gluten after a number of years of control of the rash with a strict gluten-free diet. Although there was no evidence for the antibodies having any pathogenic role in the rash of dermatitis herpetiformis, their presence may be related to the deterioration in the gluten-sensitive enteropathy.     

疱疹样皮炎误诊为药疹1例

患者女,22岁。全身泛发性红斑、水疱伴瘙痒12天。临床表现为全身对称性分布多数红斑,红斑基础上见绿豆至蚕豆大水疱,呈环状排列,尼氏征阴性。皮损组织病理示:表皮下水疱,疱底及周围真皮乳头内以中性粒细胞为主的炎性浸润。诊断:疱疹样皮炎。曾因发病前有明确服药史被误诊为药疹。     

Should all patients with dermatitis herpetiformis follow a gluten-free diet?

Aim To assess the effect of a gluten-free diet in Irish patients with dermatitis herpetiformis, and whether treatment with a gluten-free diet is as important for patients with a normal small bowel biopsy us for those with villous atrophy. Background Though a gluten-free diet is recommended in the management of dermatitis herpetiformis, many patients find it intolerably restrictive. To date we have recommended it only to patients with abnormal small bowel histology. Methods Forty patients with dermatitis herpetiformis who attended our clinic between 1979 and 1994 were studied retrospectively. Villous atrophy was present in 20 (64%) of 31 initial small bowel biopsies in patients not on a gluten-free diet. Results The median time to a 50% reduction in dapsone requirements was 6 months in patients who followed a gluten-free diet. (n = 14). 10.5 months in those who had a gluten-reduced diet (n = 4) and 10.5 months in those who took a normal diet (n = 22). Four of 14 patients (29%) on a gluten-free diet were able to discontinue medication in 1–5 years compared with 2 of 22 (9%) on a normal diet. The mean time to a 50% reduction in dapsone requirements was similar in patients with and without villous atrophy. 9.3 versus 9.0 months in patients on a gluten-free diet and 12.0 versus 15.3 months in patients on a normal diet. Conclusion We conclude that a gluten-free diet should be strongly encouraged in all dermatitis herpetiformis patients, since those with normal small bowel biopsy findings benefit equally from the diet as do those with villous atrophy.     

Impetigo herpetiformis: menstrual exacerbations for 7 years postpartum

Impetigo herpetiformis (IH) is a rare pustular dermatosis that shares striking clinical and histological similarities to generalized pustular psoriasis. The case of a 26-year-old primigravida with IH progressing in the 30th week of her pregnancy is presented. The peculiarity of our presentation relies on the reappearance of the disease postpartum. Monthly exacerbations during menstruation lasted for 7 years. Some probable clues regarding the pathogenesis of IH are suggested.