Linear IgA Bullous Dermatosis

Abstract: Linear IgA bullous dermatosis (LABD) can mimic bullous pemphigoid (BP) and/or dermatitis herpetiformis (DH) both clinically and histologically. LABD. however, can be distinguished from BP and DH by direct immunofluorescent (IF) demonstration of linear IgA. deposits along the basement membrane zone. A retrospective study of 234 cases of BP, 27 cases of LABD, 60 cases of DH, and 20 cases of cicatricial pemphigoid (CP) revealed that BP patients are significantly older than LABD or DH patients and LABD patients are significantly older than DH patients. BP and CP occur more frequently in women (65–70%) than LABD or DH (44–48%). The frequencies of C3 deposits in the basement membrane zone (BMZ) are significantly higher in BP (85%) compared with LABD (18.5%) and DH (28.3%). LABD patients varied in their response to various therapeutic agents. Some responded to corticosteroids and some to sulfones alone. whereas others required a combination of corticosteroids and sulfones.     

线状IgA大疱性皮肤病的研究进展

线状IgA大疱性皮病是一种少见的自身免疫性表皮下大疱病,其确切的发病机制还不清楚。常见的靶抗原有LAD97、LAD-1、BP180、BP230和LAD285等抗原,其中BP180抗原可能在发病中起作用。特征性的基底膜带线状IgA抗体沉积为确诊本病的主要依据。氨苯砜是治疗的首选药物。     

Linear IgA Bullous Dermatosis in a Neonate

Abstract: A newborn black boy had two facial blisters at birth that progressed to bullous lesions over the trunk, genitals, extremities, and oral and tracheal mucosa. A biopsy specimen demonstrated a subepidermal bulla with mixed eosinophilic and neutrophille, inflammatory infiltrate. Direct immunofluorescence showed linear IgA, IgG, and C3 depositions along the basement membrane zone, consistent with a diagnosis of childhood linear IgA bullous dermatosis (chronic bullous dermatosis of childhood). The skin disease was controlled with combined prednisone and dipsone. This is the youngest reported patient with the disease. Linear IgA bullous dermatosis should be considered in the differential diagnosis of blistering diseases of the newborn, and immunofluorescence should be performed on a skin biopsy specimen.     

成人线状IgA大疱性皮病1例

患者男,37岁。全身反复出现红斑、斑丘疹、水疱,伴剧痒1年半。病理检查示表皮下水疱。真皮内中性粒细胞及嗜酸性粒细胞浸润;直接免疫荧光示IgA呈线状沉积于基底膜带。皮疹特点和组织病理类似疱疹样皮炎,后根据直接免疫荧光确诊为成人线状IgA大疱性皮病,给予环磷酰胺、糖皮质激素联合治疗,取得良好效果。     

线状IgA大疱性皮病1例

患者男,15岁。全身皮肤红斑、水疱2周。皮损组织病理示表皮基底层下大疱形成,直接免疫荧光示IgA在表皮基底膜带呈线状沉积。根据临床表现、组织病理及直接免疫荧光诊断为线状IgA大疱性皮病,早期足量激素治疗可取得较好疗效。     

Childhood Linear IgA Bullous Dermatosis in Tunisia

Abstract:   The objective was to determine the demographic characteristics, the clinical features, the immuno-histological findings and response to treatment of childhood linear IgA bullous dermatosis (LABD) in Tunisia. We collected all the cases of auto-immune bullous diseases of childhood, diagnosed from January 1987 to December 2006. Based on clinical, histological, and immunofluorescent features, we identified 25 cases of LABD. Sixteen male and nine female children with a mean age of 7.5 years were identified. Clinical manifestations were characterized by a vesiculo-bullous eruption in all cases associated with mucous membrane involvement in two cases. Dapsone was the main therapy in 19 cases, associated with systemic corticosteroids in eight cases. Exclusive antibiotic therapy was successful in five cases. Sixteen of those patients had resolution of disease after a mean period of 15 months and eight patients had severe clinical presentation and required a prolonged follow-up. Childhood LABD is the most frequent bullous dermatosis in Tunisia. The majority of our patients responded rapidly to dapsone treatment and were stabilized for long time. Our cases were characterized by a minimal mucosal involvement and favorable outcome. Treatment with antibiotherapy was interesting. Erythromycin and oxacillin may be considered as an alternative therapy.     

Linear IgA Bullous Dermatosis Mimicking Erythema Multiforme in Adult

This report describes a 49-year-old woman with an erythema multiforme--like rash and direct immunofluorescence showing linear IgA deposits at the dermoepidermal junction. Light microscopy revealed features of bullous pemphigoid, dermatitis herpetiformis, and erythema multiforme; immunoelectron microscopy demonstrated IgA deposition beneath the lamina densa about anchoring fibrils. These data provide additional information about the variable clinical and histologic manifestations of the adult linear IgA bullous dermatosis and emphasize the diagnostic dilemmas of light microscopy, which are resolved by immunohistochemical methods.     

线状IgA大疱性皮病15例分析

目的分析线状IgA大疱性皮病的临床特点、治疗及预后,提高临床医生对本病的认识。方法回顾性分析本科2004年1月-2011年12月诊治的15例线状IgA大疱性皮病的临床资料。结果男6例,女9例;儿童型5例,成人型10例;皮损初始部位:面部4例,口腔黏膜及双下肢各3例;累及躯干和四肢12例,14例临床表现为红斑或正常皮肤上出现水疱,6例水疱呈环形或半环形排列。皮损组织病理均见表皮及复层鳞状上皮下水疱或裂隙,直接免疫荧光均表现为基底膜带线状IgA沉积。13例予糖皮质激素治疗,皮损均控制;2例予氨苯砜治疗,均发生不良反应。结论线状IgA大疱性皮病的皮损以口腔黏膜、面部及下肢首发居多,好发于躯干和四肢,临床表现为红斑或正常皮肤上出现水疱,呈孤立、环形和/或半环形排列;组织病理可见表皮或复层鳞状上皮下水疱或裂隙,直接免疫荧光示基底膜带线状IgA沉积是诊断的主要依据。治疗上可选用氨苯砜、糖皮质激素和磺胺类药物等。     

儿童线状IgA大疱性皮病1例

患儿男,2岁9个月。面颊、背部、双下肢泛发红斑、水疱伴疼痛15天。皮肤科情况:双侧面颊、耳廓可见红斑、糜烂,部分结痂。双下肢遍布黄豆大水疱,疱壁紧张,疱液清亮,尼氏征(-)。皮损组织病理示表皮下水疱形成,疱内可见浆液、中性粒细胞、淋巴细胞及嗜酸性粒细胞。免疫荧光IgA(+),基底膜可见线状沉积。诊断:儿童线状IgA大疱性皮病。     

线状IgA大疱皮病8例分析

线状IgA大疱皮病是一种少见的自身免疫性大疱性皮肤病 ,近 10年在我科的临床患者中 ,通过临床表现、皮肤病理检查及免疫荧光检查明确诊断为线状IgA皮病并长期随访的患者仅 8例 ,男 5例 ,女 3例 ;儿童型 5例 ,成人型 3例。 7例患者使用DDS治疗 ,皮疹得到完全控制 ,无不良作用发生。DDS可作为治疗线状IgA大疱皮病的首选药物。     

线状IgA大疱性皮病合并Graves病1例

患者女,28岁。背部反复出现水疱,伴瘙痒5个月,加重并泛发至全身4天,病程中同时出现甲状腺功能亢进。皮损组织病理和直接免疫荧光检查均提示线状IgA大疱性皮病。皮肤病和甲状腺疾病分别经糖皮质激素和同位素治疗得到缓解。     

Piperacillin-Tazobactam-Induced Linear IgA Bullous Dermatosis Supported by a T-Cell Activation Assay

Linear immunoglobulin (Ig) A bullous dermatosis (LABD) is a rare subepidermal autoimmune blistering disease characterized by linear IgA deposits at the basement membrane zone visualized with direct immunofluorescence (DIF). Most cases of LABD are idiopathic, but some are drug-induced with vancomycin being the most common causative agent. We herein report a patient presenting with blisters and erosive lesions, primarily in the intertriginous and flexor areas, consistent with a diagnosis of piperacillin-tazobactam-induced LABD based on the patient''s clinical course and histopathology, DIF, and in vitro T-cell activation assay (TAA) findings. Only one case of piperacillin-tazobactam-induced LABD has been previously reported. In addition to its rarity, our case was also unique in that the skin lesions occurred in the intertriginous and flexor areas, uncommon locations for typical adult patients with LABD, and TAA strongly suggested an association with the causative drug.     

诺氟沙星致成人线状IgA大疱性皮病1例

患者男,45岁,全身红斑伴痒1个半月。患者既往体健,无药物过敏史,家族史无特殊。皮肤科情况:躯干、四肢大小不等的地图形、环形红斑,界限清楚,部分融合成片,部分红斑边缘堤状隆起,其上可见密集分布小米粒大小串珠状排列水疱,部分破溃结痂,尼氏征阴性,黏膜未累及。根据组织病理及直接免疫荧光诊断为成人线状IgA大疱性皮病。     

成人线状IgA大疱性皮病5例临床分析

目的：了解5例成人线状IgA大疱性皮病的临床特点,以提高对该病的认识。方法：对5例成人线状IgA大疱性皮病的临床资料、组织病理、免疫荧光进行分析,并对相关文献进行复习。结果：5例患者中男3例,女2例,年龄在66—87岁之间,均表现为在红斑基础上的水疱,或外观正常的皮肤上出现的水疱,病理组织活检和免疫荧光确诊为成人线状IgA大疱性皮病。结论：成人线状IgA大疱性皮病好发年龄为〉60岁的老年人,皮疹表现类似大疱性类天疱疮、疱疹样皮炎,多数兼有两病的特点,容易误诊,直接免疫荧光检查发现沿基底膜带有均质型线状IgA沉积具有诊断价值。     

A Case of Linear IgA Bullous Dermatosis with Atypical Clinical and Ultrastructural Manifestations

We report a case of linear IgA bullous dermatosis which presented with atypical clinical and ultrastructural findings. The patient initially manifested small, subepidermal blisters scattered only in the seborrheic region. On follow-up, grouped vesicles developed in a circular form similar to dermatitis herpetiformis. They subsided after treatment with diaminodiaphenyl sulphon. Immunofluorescent study showed predominant linear IgA deposits accompanied with IgG and C3 along the basement membrane zone. These deposits existed at the floor of the blister. Ultrastructurally, immunoreactants were demonstrated beneath the lamina densa while separation occurred at lamina lucida.     

线性IgA大疱病基底膜带自身抗体的靶抗原异质性

目的 了解线性ＩｇＡ大疱病基底膜带自身抗体（ＢＭＺ－Ａｂ）靶抗原及其与其它表皮下自身免疫性大疱病相关抗原的关系。方法 有热分离法提取真表皮抗原,免疫印迹检测１１例线性ＩｇＡ大疱病中ＩｇＡ型和ＩｇＧ型ＭＢＺ－Ａｂ靶抗原的分布情况。结果 １１例线性ＩｇＡ大疱病患者中９例血清ＩｇＡ型ＭＢＺ－Ａｂ可分别与真、表皮抗原结合,其中７例结合９７０００表皮抗原（ＬＡＢＤ９７）。２例同时结合１８０００抗原,另有２例     

Linear IgA Bullous Dermatosis of Childhood with Autoantibodies to a 230 kDa Epidermal Antigen

Abstract: Linear IgA bullous dermatosis (LABD) is an autoimmune, subepidermal disease defined on the basis of direct immunofluorescence findings. However, more recent techniques used to study bullous dermatoses suggest that LABD may be heterogeneous. A patient with LABD of childhood (chronic benign disease of childhood, CBDC) was studied by indirect immunofluorescence on salt-split skin and by Western blot in an attempt to characterize the involved autoantigen. This young girl's periorificial (mouth, genitalia), erythematovesicular lesions were diagnosed initially as herpes simplex. Histologic examination revealed eosinophilic spongiosis, suggesting the diagnosis of an autoimmune blistering disease. Direct immunofluorescence showed an exclusive linear IgA deposit at the dermoepidermal junction. Indirect immunofluorescence revealed circulating IgA autoantibodies that reacted with the epidermal side of saltsplit skin; these reacted by Western blot with a 230 kDa epidermal antigen, as in bullous pemphigoid. This case, fulfilling the diagnostic clinical and direct immunofluorescence criteria for LABD/CBDC, seems to represent IgA bullous pemphigoid. It further underscores the nosologic heterogeneity of LABD, which probably includes, apart from bullous pemphigoid, epidermolysis bullosa acquisita and cicatricial pemphigoid.     

Linear IgA Bullous Dermatosis with Circulating IgG Autoantibodies to the 230 kD Epidermal Antigen

The patient was a 54-year-old woman with wide-spread bullous lesions on her trunk and oral mucosa. Histologic examination revealed a subepidermal blister with infiltration of neutrophils and eosinophils. Direct immunofluorescence showed an exclusively IgA deposition at the basement membrane zone (BMZ). Indirect immunofluorescence showed that the blister fluid, but not the serum, contained IgG antibodies against the BMZ antigen on the epidermal side of salt-split skin. Using immunoblot analysis with normal human epidermal extracts, both serum and blister fluid reacted with the 230 kD epidermal antigen. Using colloidal gold and direct immunoelectron microscopy, IgA deposition was detected in the lamina lucida. Clinically, the skin lesions responded well to dapsone. We diagnosed this case as linear IgA bullous dermatosis (LABD) with IgG class circulating autoantibodies against the epidermal 230 kD antigen. These antibodies were considered to be secondary to the damage to the epidermal basal keratinocyte in this case.