新生儿线状IgA大疱性皮病

患儿男, 生后10 d, 因皮肤红斑、水疱6 d就诊入院。皮肤科检查:全身皮肤散在或融合分布红斑, 在正常皮肤或红斑基础上可见大小不等的紧张性水疱, 部分水疱破溃、糜烂;口腔黏膜可见血疱、红色糜烂面。组织病理检查示表皮下水疱, 疱内可见中性粒细胞及少量嗜酸性粒细胞。直接免疫荧光检查显示:沿基底膜带有均质型线状IgA和颗粒状C3沉积, IgG阴性。诊断:新生儿线状IgA大疱性皮病。给予营养支持、防感染等综合治疗后, 皮肤红斑、水疱消退, 黏膜损害减轻出院。患儿出院后16个月电话随访, 一般情况良好, 皮肤黏膜皮损消退、愈合, 无新发皮疹, 生长发育正常。     

儿童大疱性类天疱疮

报告1例儿童大疱性类天疱疮.患儿男,18个月.2个月前躯干和双下肢皮肤出现紧张性水疱、大疱,尼氏征阴性,外用糖皮质激素治疗效果不佳.皮损组织病理检查示表皮下水疱;卣接免疫荧光示:lgG、C3沉积于基膜,间接免疫荧光示抗表皮基膜抗体阳性.诊断为儿童大疱性类天疱疮,予以口服泼尼松治疗后痊愈.     

大疱性类天疱疮伴食管恶性黑色素瘤1例

报道大疱性类天疱疮（BP）伴食管恶性黑色素瘤（PMME）1例。患者女,61岁,既往PMME史3年余,现黏膜破溃4个月,加重伴周身红斑水疱0.5个月。皮肤科检查：面部出现水肿性红斑,红斑基础上覆厚腻黑痂;躯干、四肢泛发水肿性红斑、丘疹,红斑及正常皮肤基础上可见粟粒至甲盖大小水疱,疱壁紧张,疱液清亮,部分水疱破溃渗出结痂,尼氏征阴性;口腔可见溃疡;外阴部黏膜糜烂。组织病理及免疫荧光检查均符合BP。诊断：BP伴发PMME。     

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。     

大疱性疥疮

报告1例大疱性疥疮。患者男,65岁。3个月前无明显诱因躯干、四肢出现散在红斑、水疱、糜烂、结痂,伴瘙痒。患者1个月前指缝间、阴囊处出现类似红斑、丘疹,瘙痒明显。皮肤科检查:躯干、四肢可见弥漫性红斑,部分红斑上可见紧张性水疱,疱壁紧张,疱液清亮,部分水疱破裂后糜烂,可见红色糜烂面,部分表面结痂;双手指缝间、阴囊散在红斑,苔藓样变,可见抓痕。皮损组织病理检查:(左股内侧皮损)角化不全,浆液渗出,可见表皮下疱,疱内可见浆液,大量嗜酸性粒细胞及红细胞,真皮浅层可见嗜酸性粒细胞及中性粒细胞浸润;直接免疫荧光:皮肤全层未见特异性荧光,间接免疫荧光:真皮与表皮交界处(-);镜检发现疥螨。诊断:大疱性疥疮。     

成人线状IgA大疱性皮病2例

报道2例成人线状IgA大疱性皮病。患者1,女,57岁。躯干、四肢红斑、水疱2个月,伴痒。皮肤科检查:躯干、四肢水肿性红斑,呈环形,部分边缘可见黄豆粒大水疱,疱液清,尼氏征阴性。患者2,女,43岁。躯干、四肢散发性水疱20天,伴痒。皮肤科检查:躯干、四肢绿豆至黄豆大水疱,疱液清,尼氏征阴性。两例患者组织病理检查均表现为:表皮下水疱,真皮乳头嗜中性粒细胞小脓肿;直接免疫荧光示:表皮基底膜带IgA线状沉积。     

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。     

儿童慢性大疱性皮肤病——一种小水疱性变型(线状IgA皮肤病)

作者报告一例3岁黑人女孩,面部及四肢反复出现瘙痒性0.3cm的小水疱1个月。从未出现过大疱,无腹泻、体重减轻或营养吸收障碍,无家族史。体格检查:面部及四肢(主要是伸侧)有散在不成群的小水疱,同时可见新损害和结痂的不同阶段的损害。愈合后不留疤痕,但有色素沉着。未见玫瑰花环或宝石串样改变,水疱细菌培养及病毒分离均阴性。损害或损害周围活检显示:表皮下水疱形成。疱内细胞主要为嗜中性细胞,偶见嗜酸性细胞,可见核尘和纤维蛋白束。乳头内有嗜中性白细胞聚集。直接免疫荧光(DIF)检查:在基底膜带(BMZ)显示线状IgA沉积,间接免疫荧光(IIF)检查阴性。开始用氮苯砜25mg/d治疗2个月,以后隔日25mg,全疗程持续9个月,10天内病变完全消退,突然停药无新损害发生。随访4个月疾病无活动性。讨论:本病临床表现是在正常皮肤或红斑的基     

中性粒细胞沿基底层呈线状排列的大疱性类天疱疮一例

患者男,71岁。全身反复发生红斑3个月,出现水疱1周,伴剧痒。患者3个月前躯干、四肢陆续出现浮肿性红斑伴瘙痒,一度按湿疹治疗皮疹仍反复发作,时轻时重。就诊前1周在正常皮肤或红斑基础上发生水疱,破溃后易结痂。患脑卒中8年。家族中无类似皮肤病史。体检：右侧肢体偏瘫。余各系统检查未见异常。皮肤科检查：躯干、四肢散在大小不一的浮肿性红斑,部分红斑上可见水疱、大疱、糜烂及结痂。水疱Nikolsky征阴性,疱液为透明浆液性（图1）……     

儿童型线状IgA大疱性皮病一例

患儿女,7岁,主因全身起水疱伴剧痒1月余入院。皮科情况：全身见较多红色斑片、丘疹、丘疱疹及水疱,部分水疱呈腊肠样,水疱主要分布于红斑边缘。水疱破后形成糜烂伴结痂。以面、臀及股内侧较密集,部分表面可见浆痂。病理：表皮下水疱,疱内可见散在嗜中性粒细胞,疱底及疱两侧表皮可见大量嗜中性粒细胞,偶见嗜酸性粒细胞。免疫荧光检查：DIF：IgA 弱阳性,IgG 阳性,IIF：IgA 阴性。诊断：儿童型线状IgA大疱性皮病。治疗：氨苯砜,100mg/天,10余天后基本痊愈,至今40余天,皮疹无复发。讨论：线状IgA大疱性皮病(LABD)由Chorzelski ( 1979)首先命名。本例患儿发病早、具特征性皮损,HE病理：表皮下水疱,疱内可见散在嗜中性粒细胞,疱底及疱两侧表皮可见大量嗜中性粒细胞。免疫荧光可见IgG、IgA于基底膜带呈线状沉积。氨苯砜治疗效果好,儿童型线状IgA大疱性皮病诊断成立。本例免疫病理IgA、IgG均阳性,提示该患儿是否可能存在IgA大疱性皮病与大疱性类天疱疮的重叠,还有待于进一步检查。     

Reconstitution of structure and cell function in human skin grafts derived from cryopreserved allogeneic dermis and autologous cultured keratinocytes

Grafts of allogeneic dermis plus autologous epidermal cell cultures were used to replace extensively burned skin. Cryopreserved split-thickness cadaveric skin was grafted onto debrided burn wound, and autologous keratinocytes were cultured from uninjured donor sites. Several weeks later, allograft epidermis was abraded and replaced with the keratinocyte cultures. The final grafts were thus composites of autologous cultured epidermis and allogeneic dermis. In a case with 28 months follow-up, reconstitution of the dermal-epidermal (BMZ.1) and microvascular (BMZ.2) basement membrane zones was studied immunohistochemically and ultrastructurally. Immediately before grafting, thawed cryopreserved skin reacted with antibodies against laminin and type IV collagen in normal patterns. Twenty-nine days after grafting, BMZ.1 reacted weakly with both antibodies, and anticollagen type IV reactivity was absent from BMZ.2. Antilaminin reactivity of BMZ.2, however, was moderately intense, consistent with recent neovascularization. On day 29, the allograft epidermis was replaced with autologous keratinocyte cultures. Twenty-five days later (54 d after allografting), staining of both BMZs was intense with both antibodies. Ultrastructurally, at day 76 (47 d after culture placement) BMZ.1 revealed only small hemidesmosomes, few incipient anchoring fibrils, and a discontinuous lamina densa. BMZ.2, however, was fully reconstituted. By 124 d, both BMZs appeared normal. Observations in the dermis at 76 d included the presence of lymphocytes, organellar debris, and hyperactive collagen fibrillogenesis, all indicative of dermal remodelling. The microvasculature was well differentiated, but no elastic fibers or nerves were found. In the epidermis, melanocytes and evidence of melanosome transfer were seen at 5, 47, and 95 d after grafting of keratinocyte cultures. We conclude that the composite procedure reconstitutes skin with excellent textural and histologic qualities.     

Direct immunofluorescence in lesional and uninvolved skin in dle

Direct immunofluroscence was evaluated in 16 patients with DLE including 1 case of lupus profundus and 4 with disseminated DLE. A BMZ band with multiple immunoreactants was demonstrated in lesional skin in 14 patients (87.5%), while 2 had only single immunoreactants. C3 and lgM were the commonest reactants followed by lgG, lgA and fibrinogen. A perifollicular prominence was seen in several patients. Four patients showed a band in uninvolved skin. This may indicate a potential to develop SLE. ANA was also positive in 5 patients but ds DNS was negative.     

Coexistence of psoriasis and linear IgA bullous dermatosis

Linear IgA bullous dermatosis (LABD) is characterized by IgA autoantibodies against components of the basement membrane zone (BMZ). A 97-kDa protein is one of the major autoantigens associated with this disease. We report a 68-year-old man who developed LABD after a 3-year history of psoriasis and in the context of active hepatitis C virus infection. He had been treated with cyclosporin for psoriasis for about 9 months. Histologically, there was a subepidermal blister containing neutrophils and eosinophils with lymphocytes infiltrating predominantly in the dermis. Direct immunofluorescent staining showed linear IgA deposition at the BMZ. The patient's IgA autoantibodies bound exclusively to the epidermal side of 1 mol/L salt-split normal human skin. Immunoblot analysis identified a 97-kDa autoantigen in epidermal extracts. This appears to be the first case of LABD with IgA autoantibodies against a 97-kDa autoantigen, associated with psoriasis and hepatitis C virus infection.     

Comparison between the expression of basement membrane zone antigens of human interfollicular epidermis and anagen hair follicle using indirect immunofluorescence

BACKGROUND: The composition of the basement membrane zone (BMZ) or dermal-epidermal junction in the interfollicular skin has been well documented. However, little is known about the BMZ or connective tissue-epithelial junction along the hair follicle. OBJECTIVES: To determine whether the BMZ antigens in the interfollicular epidermis are also present in the BMZ of the anagen hair follicle and to compare whether the expression and distribution of the BMZ components vary between the interfollicular epidermis and the anagen follicle and within different regions of the hair follicle. METHODS: Longitudinal cryostat sections of scalp margin specimens from four adult patients undergoing cosmetic surgery, and without known pathology were stained with a panel of monoclonal and polyclonal antibodies to different BMZ constituents using standard indirect immunofluorescence. RESULTS: All the BMZ antigens found in the normal interfollicular epidermis were expressed in the anagen follicle; however, there were regional variations in the intensity and patterns of fluorescence. All the antigens were expressed in a continuous linear pattern along the BMZ of the interfollicular skin, the infundibulum, and the middle part of the hair follicle. Differences were observed in the lower follicle and the hair bulb. There was continuous expression throughout the BMZ of the follicle of laminin-1 and collagen IV, but in contrast, expression of other antigens decreased down the lower follicle. There was weak or even negative staining with antibodies to alpha 6 beta 4 integrin, laminin-5, anchoring filaments, and type VII collagen in the outer aspect of the bulb compared with the hair papilla. In addition, there were special patterns observed along the bilateral middle and lower follicle. CONCLUSIONS: Despite the common embryological origin between the interfollicular epidermis and the hair follicle, there is variation in the expression of the BMZ antigens. This may be explained by the histological specialization and functional requirements that reflect the dynamic hair growth cycle.     

Demonstration of antibody to 230 kDa bullous pemphigoid antigen in lichen planus-like keratosis.

We describe a 67-year-old man with lichen planus-like keratosis associated with anti-230 kDa bullous pemphigoid antigen (BPAG1) autoantibody. The patient had noticed solitary dark brown macule more than 6 years previously on his left chest. Histological findings showed hypergranulosis, irregular acanthosis, liquefaction degeneration of basal cells, band-like infiltration of lymphocytes at the subepidermal portion, and a cleft at the basement membrane zone (BMZ), resulting in the formation of subepidermal blisters. Direct immunofluorescence findings of perilesional skin showed a linear deposition of IgG at BMZ. On indirect immunofluorescent study using normal human skin, circulating IgG autoantibody to BMZ was present in the patient's serum at a titer of 1:80. The antigen located on the epidermal site of normal skin split by 1M NaCl was reacted with the patient's serum. Immunoblot analysis using epidermal extracts demonstrated the presence of IgG antibody directed to BPAG1 in the patient's serum. These observations suggest that the presence of an antibody to BPAG1 could be caused by the damage of basal cells following lichen planus-like keratosis.     

KF-1 monoclonal antibody defines a specific basement membrane antigen defect in dystrophic forms of epidermolysis bullosa

The monoclonal antibody, KF-1, identifies a noncollagenous constituent of the lamina densa of the basement membrane zone (BMZ) of skin. In order to determine whether this BMZ constituent is affected in epidermolysis bullosa (EB), a mechanobullous skin disease often resulting in marked disfigurement, we have examined skin from patients with various forms of this disease for binding by KF-1 as well as for binding by polyclonal antibodies to laminin, type IV collagen, and bullous pemphigoid antigen, three other known BMZ components of normal skin. In all specimens from patients with simplex and junctional forms of EB, all four antibodies bound normally. In contrast, absent or diminished KF-1 binding was noted in all skin specimens from patients with dystrophic EB; antibodies directed against the other BMZ constituents, however, bound normally. This suggests that KF-1 may play a role in the structural integrity of normal skin and its absence or diminution may be important in the pathogenesis of lesion formation in dystrophic EB.     

Alterations of basement membrane zone and cutaneous microvasculature in morphea and extragenital lichen sclerosus

The aim of this study was to compare alterations of the basement membrane zone (BMZ) and to visualize changes within the skin vascular network in morphea and extragenital lichen sclerosus with the use of laser scanning confocal microscopy. This work was performed in eight plaques of morphea (three active and five inactive) and eight of lichen sclerosus (three of short duration and five long-lasting). Biopsy specimens from six healthy individuals served as controls. The biopsies were cut into 40-microm-thick sections, labeled with antibodies against beta4-intergin (a lamina lucida marker), collagen IV, and the N-terminal end of collagen VII (lamina densa markers) and C-terminal end of collagen VII (a sublamina densa marker) and studied using laser scanning confocal microscopy. Three-dimensional reconstruction of various regions of the BMZ showed a decreased number and size of the dermal papillae both in morphea and lichen sclerosus compared with normal skin. In morphea, the continuity of the BMZ at the level of lamina lucida, lamina densa, and sublamina densa was preserved whereas in LS numerous invaginations and holes were present in the BMZ at the level of the lamina lucida and lamina densa. Thus the alterations of the BMZ in morphea differ from those in lichen sclerosus. Three-dimensional reconstruction of the skin vascular network showed increased angiogenesis only in the early inflammatory stage of morphea, whereas in inactive morphea and lichen sclerosus various numbers of enlarged vessels were visible. The changes in the vascular network in morphea appear to be related to the activity of the disease.     

获得性大疱性表皮松解症的诊断—1M NaCl分离皮肤法

本文报导用间接免疫荧光技术,以1MNaCl分离正常皮肤为底物,其分离部位在透明板,筛选128份含有抗基底膜抗体IgG的疱病血清,以确定其中有多少为EBA,结果102例血清为表皮型,9例为联合型(表皮和真皮均着色),8例为真皮型,9例为阴性.结果显示诊断为类天疱疮患者中大约有10%为EBA,同时详细介绍了皮肤分离法.     

Alterations of basement membrane zone in bullous and non-bullous variants of extragenital lichen sclerosus

The aim of the study was to compare alterations of various regions of the basement membrane zone (BMZ) in lichen sclerosus (LS) using laser scanning confocal microscopy. The study included three cases of bullous LS, one case of bullous LS that developed in the course of graft-versus-host disease (GVHD), and six cases of non-bullous LS. Three cases of morphea served as a control. Biopsies from patients' skin and control biopsies from normal human skin were cut into 30-microm thick slides and labeled with antibodies against beta4-intergin (lamina lucida marker), collagen IV, and the N-terminal end of collagen VII (lamina densa markers) and the C-terminal end of collagen VII (sublamina densa marker) using routine immunofluorescence (IF). Three-dimensional (3D) reconstruction of various regions of the BMZ showed a decrease in the number and size of the dermal papillae in LS and morphea as compared with normal skin. In LS numerous invaginations and holes were present in the BMZ at the level of the lamina lucida and lamina densa. Computer animation of 3D projections revealed that the thickness of the lamina densa observed under the light microscopy is an optical artifact dependent on periodical tortion of the lamina densa along its axis. Torsions and invaginations of the BMZ are equally responsible for the phenomenon of artificial reduplication of the lamina densa observed at the ultrastructural level. IF labeling with antibody against the N-terminal end of collagen VII disclosed the presence of a large hole (up to 25 microm) in the lamina densa and the presence of granular material in deep dermis suggestive of partial degradation of lamina densa at the level of anchoring fibers. An IF mapping study showed blister formation below the lamina densa in three patients with bullous LS, whereas in a case of LS associated with GVHD, a blister formed through the basal layer of the epidermis. In morphea, there was flattening of BMZ at the level of lamina lucida, lamina densa, and sublamina densa but the continuity of BMZ was preserved. Three-dimensional reconstruction of dermal-epidermal junction in LS revealed alterations of the BMZ, most pronounced at the level of the lamina densa and sublamina densa.     

Basement membrane zone and dermal extracellular matrix of the vulva, vagina and amnion: An immunohistochemical study with comparison with non-reproductive epithelium

Background/Objectives: The basement membrane zone (BMZ) is an anatomically defined region present in all types of skin and mucosa, linking the epithelium to the mesenchyme with a complex structure to provide adhesion. Altered antigenic expression of the BMZ is implicated in interface dermatoses, and the BMZ is targeted by autoantibodies in subepidermal immunobullous dermatoses. This study aims to compare the antigenic expression of the BMZ and the dermal extracellular matrix in female genital skin and mucosa and amnion, with non‐reproductive skin and mucosa. Methods: An indirect immunofluorescence technique was used to compare the antigenic expression of hemidesmosome, lamina lucida, anchoring filaments, lamina densa, anchoring fibrils and extracellular matrix in samples of non‐reproductive skin (three), oral mucosa (three), vulval skin (two), vagina (three) and amnion (four). Results: Antigenic expression was similar in the stratified epithelium of reproductive and non‐reproductive skin and mucosa, but differed in the simple cuboidal epithelium of amnion, which had reduced expression of dermal‐associated antigens. Conclusions: The BMZ and dermal extracellular matrix of vagina and vulva are very similar to those of non‐reproductive skin and mucosa despite their various functions, but differs from amnion. Their antigenic expression does not fully account for the anatomical distribution of immunobullous and interface dermatoses.