皮肤异色病样淀粉样变性免疫荧光及电镜观察

报告1例皮肤异色病样淀粉样变性.患者男,40岁.四肢、躯干色素沉着和色素减退相间,伴苔藓样丘疹及毛细血管扩张20年,无自觉症状.全身皮损于夏季加重且可出现水疱.家族中无类似疾病史,父母系近亲结婚(表兄妹).皮损组织病理检查示真皮乳头内有红色团块样均质性物质,结晶紫染色阳性.直接免疫荧光(DIF)检查:表皮基膜带及表皮细胞间IgG、IgM、IgA、C3均阴性,真皮乳头及真皮浅层可见大量团块状物质沉积,其中IgG标记可见较强荧光.电镜检查:真皮乳头及真皮浅层可见大量团块状物质沉积与基膜带及基底细胞关系密切.     

皮肤黏蛋白病

报告1例皮肤黏蛋白病.患者男,19岁.背部、右上肢结节半年余.皮肤科检查见背部有3枚直径2.5～9.5 cm的斑块,右上臂伸侧有1枚直径5 cm的皮肤结节,质地中等有弹性,边界欠清,无红肿及破溃,无压痛.皮损组织病理检查示:真皮胶原稀疏,血管、汗腺、毛附属器周围可见致密的单-核细胞浸润.阿新蓝染色示:真皮、皮下、毛囊周嗣均见染淡蓝色物质.直接免疫荧光示:免疫球蛋白IgM、补体C3基膜带呈线状沉积,免疫球蛋白IsG、IgA阴性.电镜检查示:胶原束间出现团状电子密度较高的颗粒状、纤维状物质沉积,未见血管病变.符合皮肤黏蛋白病.     

伴抗基底膜自身抗体的皮肤异色症样淀粉样变病1例

报道1例伴抗基底膜自身抗体阳性的皮肤异色症样淀粉样变病,并对相关文献进行复习.患者女,22岁.面颈部、躯干、四肢出现瘙痒性红斑、水疱及弥漫性网状色素沉着性斑片8年余.皮损组织病理示：表皮角化过度,基底细胞液化变性,真皮乳头均一化物质沉积,结晶紫染色阳性;直接免疫荧光（DIF）示：基底膜带及乳头IgG、IgM、IgA和C3沉积.     

神经皮肤黑变病

报告1例神经皮肤黑变病.患儿男,2 个月.全身多发褐色至黑色斑块2 个月,抽搐1 个月.皮损组织病理示先天性色素痣表现;免疫组织病理:S-100 蛋白阳性,HMB-45 弱阳性;脑电图示:癫样放电;头颅磁共振成像(MRI)示:双侧侧脑室扩大,小脑延髓池扩大,第四脑室扩大,脑积水,考虑为Dandy-Walker 畸形.诊断为神经皮肤黑变病.予以肌内注射苯巴比妥等对症治疗.     

皮肤中型原藻病

报告1例中型原藻病.患者女,26岁.14年前,右侧面部出现红斑、丘疹、鳞屑,皮损逐渐扩大融合成片.组织病理检查（PAS染色）示,表皮及真皮浅层可见厚壁孢子,直径8～12μm,内有数量不等的内孢子.于单含氯霉素的沙堡琼脂上培养,长出奶油色乳酪样菌落,镜下可见大量直径10～18 μm的圆形、内有直径3～8μm内孢子的厚壁孢子,经放线菌酮抑制试验、温度试验、生化特性试验及形态学特征等初步鉴定为中型原藻.     

发生Sweet病样皮肤损害的Behcet病12例

Behcet病是多系统损害疾病,其中皮肤损害发生早,发病率高,类型多,不仅有常见见的结节性红斑样及毛囊炎样损害,还有少见而特殊的Sweet病样皮肤损害^[1].     

皮肤异色病样淀粉样变病

皮肤异色病样淀粉样变是原发性皮肤淀粉样变的少见类型,国内外关于本病的报道较少;多种因素参与皮肤异色病样淀粉样变发病,明确诊断需组织病理的支持,必要时需行电镜观察,且需与有皮肤异色样变表现的其他疾病相鉴别.基于已报道的病例,就该病病因、临床特点、组织病理、诊断及鉴别诊断等方面进行探讨.     

结节性皮肤淀粉样变病1例

患者女,48岁。右腋下大小不等的结节样斑块十余年,皮损表现为淡黄色、质硬的斑块和结节。组织病理学检查示:表皮下有不规则的胶样物质沉积。刚果红染色阳性。诊断:结节性皮肤淀粉样变病。     

皮肤异色病样淀粉样变病

皮肤异色病样淀粉样变是原发性皮肤淀粉样变的少见类型,国内外关于本病的报道较少;多种因素参与皮肤异色病样淀粉样变发病,明确诊断需组织病理的支持,必要时需行电镜观察,且需与有皮肤异色样变表现的其他疾病相鉴别.基于已报道的病例,就该病病因、临床特点、组织病理、诊断及鉴别诊断等方面进行探讨.     

皮肤Rosai-Dorfman病1例

患者男,28岁。左小腿内侧暗红色结节4月。皮损组织病理示:示真皮全层及皮下组织中有大量组织细胞浸润,其间有淋巴细胞及浆细胞,并可见组织细胞内吞噬淋巴细胞及浆细胞。免疫组化结果示淡染的组织细胞S-100蛋白阳性、CD68阳性。诊断:皮肤Rosai-Dorfman病。     

Waldenstr?m macroglobulinemia with an IgM-kappa antiepidermal basement membrane zone antibody.

BACKGROUND--Waldenstr?m macroglobulinemia, a lymphoplasmacytoid cell malignant neoplasm associated with a monoclonal IgM paraprotein, has been associated with a number of cutaneous manifestations. On rare occasions, IgM deposits have been demonstrated in the epidermal basement zone of patients with WM. OBSERVATIONS--We report the case of a patient with Waldenstr?m macroglobulinemia and IgM-kappa paraprotein who had development of an eruption of pruritic papules and demonstrated the following unusual immunopathologic findings: (1) deposits of IgM-kappa in the epidermal basement membrane zone of lesional and nonlesional skin; (2) a circulating IgM-kappa antiepidermal basement membrane zone antibody; and (3) binding of this circulating IgM-kappa antiepidermal basement membrane zone antibody to both sides of 1 mol/L sodium chloride split skin. The cutaneous eruption cleared completely with oral psoralen with long-wave UV radiation in the A range (PUVA) therapy. CONCLUSIONS--We present a patient with Waldenstr?m macroglobulinemia who had a distinctive papular eruption and immunopathologic findings suggesting that his paraprotein has specificity for the epidermal basement membrane zone.     

Immunopathological studies in herpes gestationis

Four cases of herpes gestationis are reported and the immunopathological findings in these patients described. Complement deposition at the basement membrane zone of the patients' peri-bullous skin was seen in all patients, immunoglobulin deposition in two. A circulating factor capable of fixing complement on the basement membrane zone of normal human skin was present in three patients. These findings are discussed and compared with the immunopathological findings in bullous pemphigoid.     

Immunofluorescence on split skin for the detection and differentiation of basement membrane zone autoantibodies.

The autoimmune subepidermal bullous diseases are characterized by autoantibodies to the basement membrane zone of stratified squamous epithelium. Recent studies have shown that the antibodies have characteristic ultrastructural and antigenic binding properties and that differentiating between those properties can be useful in distinguishing one disease from another. Immunofluorescence microscopy is widely used to detect basement membrane zone autoantibodies. The test has traditionally used tissue substrates with an intact basement membrane zone. Those substrates are limited because autoantibody binding cannot always be detected and because autoantibodies with different ultrastructural and antigenic binding properties cannot be distinguished from each other. Normal human skin that has been separated through the basement membrane zone (i.e., split skin) has recently been used as a substrate for detecting and characterizing basement membrane zone autoantibodies by immunofluorescence. Studies indicate that split skin is a more sensitive substrate than intact skin for detecting the antibodies and that antibodies with different ultrastructural binding sites can often be differentiated from one another on split skin. Those studies suggest split skin is the substrate of choice for the routine immunofluorescence evaluation of autoimmune subepidermal bullous diseases.     

Pityriasis Rosea in a Patient with Epidermolysis Bullosa Dystrophica

A patient with clinical and histologic features of epidermolysis bullosa dystrophica-recessive (EBD-R) developed superimposed clinical lesions of pityriasis rosea (PR). Electron microscopy showed distinct "blebbing" of basal cells at the basement membrane zone in clinically normal skin of a non-predilected area, as well as in scarred skin from a predilected area and a lesion of PR. An increased susceptibility of the basement membrane zone to proteolytic enzymes is suggested as a possible hypothesis to explain these observations.     

The activation of antigens of the basement membrane zone by proteolytic enzymes in vitro.

Following brief exposure of cryostat sections of human skin to the action of proteolytic enzymes (papain or trypsin), there was a pronounced increase in the antibody binding sites of the basement membrane zone, shown in indirect immunofluorescence by an increased intensity in fluorescence of the basement membrane zone as compared with preparations which had not previously undergone incubation, and by an increase of 5-6 (papain) or 4-5 (trypsin) titre dilution steps. This effect was practically absent when guinea-pig tongue was used as the antigenic substrate. In conjunction with findings published in the literature, our results can be interpreted as indicating that the activation of the basement membrane zone antigens by proteolytic enzymes is associated with an increase in antigenicity which results in the formation of "auto-antibodies" of the basement membrane zone antibody type.     

A Case of Linear IgA Disease: An Immunofluorescent Study Using Confocal Laser Scan Microscopy

A 79-year-old Japanese woman presented with erythema and bullae on her trunk and limbs. Histological examination of the skin lesions showed subepidermal bullae and polymorphonuclear leukocyte infiltration into the papillary dermis. A direct immunofluorescent study showed the linear deposition of IgA, but not of IgG or IgM, in the basement membrane zone. Indirect immunofluorescence of the serum using confocal laser scan microscopy showed IgA, but not IgG, reactivity in the basement membrane zone. In double immunostaining experiments, IgA reactivity was also observed on the epidermal side; laminin 5 was detected on the dermal side.     

The epidermal basement membrane zone--structure, ontogeny, and role in disease

Since many dermatologic diseases affect the epidermal basement membrane zone, there has been intense investigation into the role of epidermal basement membrane zone constituents in various skin diseases, particularly subepidermal blistering skin diseases. The epidermal basement membrane zone consists of four major structural components--the basal cell plasma membrane, the lamina lucida, the lamina densa, and the sublamina densa zone, which contains anchoring fibrils. The lamina lucida is composed of laminin, bullous pemphigoid antigen (a disease-specific glycoprotein identified by antibodies circulating in patients' sera), and other as yet poorly defined antigens identified by in vivo bound and circulating antibodies in the sera of patients with herpes gestationis, scarring pemphigoid, and others. The lamina densa consists of type IV collagen and KF-1 antigen, which is noncollagenous and is identified by a skin-specific monoclonal antibody. The sublamina densa zone consists of AF-1 and AF-2 antigens and the epidermolysis bullosa acquisita antigen(s). Knowledge of the structure and chemical composition of the basement membrane zone is critical to an understanding of some of the genetic and immunologically mediated blistering skin diseases.     

Bullous dermatosis and systemic lupus erythematosus in a 15-year-old boy.

A 15-year-old boy had a bullous eruption suggestive of bullous pemphigoid and established systemic lupus erythematosus (SLE). Direct immunofluorescence studies of the bullae and adjacent skin revealed the linear deposition of IgG and complement localized to the basement membrane zone. Indirect immunofluorescence examination of the serum failed to reveal circulating basement membrane zone antibodies. The differential diagnosis of the bullous eruption is reviewed, and the problem of diagnosis in cases of coexistent bullous disease and SLE is discussed.     

Chronic bullous disease of childhood and linear IgA disease of adults are IgA1-mediated diseases

Linear IgA disease is characterized by the presence of linear IgA deposits at the basement membrane zone of the skin, and in some cases by circulating basement membrane zone antibodies. The disease occurs in both adults and children, and is designated adult linear IgA disease in the former and chronic bullous disease of childhood in the latter. The subclass distribution of the circulating and bound basement membrane zone antibodies was studied in 32 children and eight adults. The results were compared with five dermatitis herpetiformis patients and five normal controls. The circulating antibodies (39 patients) and the cutaneous deposits (39 patients) were IgA1 in all 40 patients with linear IgA disease. The cutaneous deposits in dermatitis herpetiformis were also all IgA1, and no circulating antibodies were detected. The controls were all negative. This large series of children and adults with linear IgA disease demonstrates that the circulating and cutaneous basement membrane zone deposits are all IgA1, and suggests that linear IgA disease is an IgA1-mediated disease.     

二例大疱性类天疱疮皮损的透射电镜及免疫病理观察

所见二例,电镜下见棘细胞结构正常,水疱在表皮下,腔内有红、白细胞,表皮下层基底细胞间结构疏松,疱顶部基底膜消失,真皮胶原纤维结构正常。直接免疫荧光检查,见表皮下水疱内及基底膜带有IgG沉积,未见IgA沉积,于水疱内、基底膜、毛囊周围及小血管壁有C_3呈线状沉积。并初步探讨了其发生机制。