大疱性系统性红斑狼疮2例

报告2例大疱性系统性红斑狼疮,均为青年男性。以面部、躯干、四肢红斑、水疱为首发症状。例1患者白细胞总数1.85×109/L;24h尿蛋白定量1.040g/24h;ANA(++);抗SM(+);直接免疫荧光:IgG、IgA、IgM、C3基底膜荧光(+)。例2患者有踝关节疼痛;口腔溃疡;抗核抗体ANA(++);抗ds-DNA1/10阳性,抗SM(+);24h尿蛋白定量7.184g/24h。均符合系统性红斑狼疮诊断标准。两例患者皮肤组织病理结合直接免疫荧光符合大疱性系统性红斑狼疮病理改变。糖皮质激素联合氨苯砜等治疗,皮损全部消退。     

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。     

大疱性系统性红斑狼疮一例

65岁女性患者,全身红斑、水疱伴瘙痒1个月。面部、躯干、四肢散在分布暗红斑,红斑基础上可见松弛性水疱或大疱,尼氏征阴性,口腔颊黏膜散在浅表糜烂。左肩部新发红斑处皮损组织病理:表皮下裂隙、水疱形成,疱内少量嗜酸粒细胞及真皮浅层淋巴细胞、中性粒细胞浸润。直接免疫荧光可见Ig A、Ig G和补体C3在表皮下呈线状沉积。间接免疫荧光显示抗基膜带抗体阳性(滴度1:80)。左面颊皮损组织病理:表皮萎缩变薄,表真皮分离,真皮浅中层血管扩张,周围散在淋巴细胞为主的炎性细胞浸润,胶原嗜碱变性。抗核抗体(ANA)阳性(1:320);Sm抗体阳性;动态红细胞沉降率60 mm/h;补体C3 0.59 g/L,C1q 129 mg/L。诊断:大疱性系统性红斑狼疮。经甲泼尼龙联合硫酸羟氯喹治疗2周后好转出院,随访6个月无复发。     

表现为环状红斑水疱的大疱性系统性红斑狼疮一例

45岁女性患者,躯干及四肢红斑、水疱伴痒痛20余天。皮损表现为躯干、上肢对称分布大小不等的类圆形或同心圆状红斑、丘疹及水疱,尼氏征阴性;双下肢及双手足背侧可见界限清楚的水肿性红斑,口腔及生殖器黏膜未受累。抗核抗体(ANA)、SSA抗体、Ro-52抗体、抗心磷脂抗体均阳性,U1RNP/sm抗体、Sm抗体弱阳性;补体C3、C4降低;间接免疫荧光示基膜带抗类天疱疮抗体阳性。诊断:大疱性系统性红斑狼疮。给予糖皮质激素及硫酸羟氯喹治疗后病情好转。     

PD-1抑制剂治疗原发性肝癌出现大疱性类天疱疮一例

47岁男性患者,躯干、四肢皮损伴瘙痒2个月。患者1年前因原发性肝癌行信迪利单抗免疫治疗,2个月前躯干、四肢出现红斑、水疱。右下腹红斑组织病理检查示表皮下水疱,左大腿红斑直接免疫荧光示Ig G沿基膜带沉积。血清BP180抗体> 200 RU/ml。诊断：大疱性类天疱疮。予甲泼尼龙40mg/d静脉滴注,辅以卤米松乳膏外用,经治疗红斑变暗,水疱干涸、结痂。     

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。     

系统性红斑狼疮合并线状IgA大疱性皮病一例

患者女,38岁,面部蝶形红斑、关节痛5年,四肢、躯干及口腔黏膜红斑水疱,伴瘙痒1个月。5年前面部蝶形红斑、光过敏、关节痛,蛋白尿、ANA及ds—DNA阳性,于外院诊断为SLE,应用泼尼松治疗,起始剂量60mg／d,2年前减量至20mg／d,维持至今,病情平稳。1个月前无明显诱因于四肢、躯干及口腔黏膜出现红斑、水疱,伴剧烈瘙痒。疑为红斑狼疮活动,遂将泼尼松加量至50mg／d,     

大疱性系统性红斑狼疮1例

分析报告1例大疱性系统性红斑狼疮。患者以全身皮肤粘膜起红斑、水疱为表现起病，伴有口腔溃疡、双膝关节疼痛，尿蛋白3 ，ANA阳性1：160颗粒型，ds-DNA抗体阳性，ENA阳性、SS-A阳性。取躯干部皮损行组织病理活检，皿染色示：表皮下水疱，真皮浅层水肿，真皮乳头见中性粒细胞聚集，免疫病理示基底膜带可见IgG呈颗粒状沉积，符合大疱性系统性红斑狼疮病理改变。该病例为大疱性系统性红斑狼疮。     

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）……     

红皮病性大疱性类天疱疮1例

 患者女,78岁。因“躯干、四肢红斑、鳞屑伴瘙痒5年,加重3年余”入院,入院后1周大腿及腹部出现紧张性水疱。住院第6天、第9天血清抗BP180抗体均阳性,IgE>2 500 U/mL。皮损组织病理可见表皮下水疱,真皮浅层大量淋巴细胞为主的浸润。直接免疫荧光示:基底膜带IgG和C3线状沉积,IgA阴性。诊断：红皮病性大疱性类天疱疮。给予甲泼尼龙40 mg静脉滴注1天1次,治疗1周,病情好转出院。1年后随访,患者泼尼松片减量至15 mg口服1天1次,躯干四肢可见轻度红斑,无水疱、糜烂和瘙痒     

种痘水疱病样EB病毒感染相关T细胞增生性疾病一例

患者,女,7岁。皮肤出现红斑水疱伴发热,反复发作4年。下肢皮损组织病理检查示：表皮坏死,真皮血管管壁变性、坏死,血管周围淋巴细胞浸润,浸润细胞有异型。皮下脂肪组织血管管壁坏死,周围可见淋巴细胞浸润。EB（Epstein-Barr）病毒IgG抗体（EBV-CA-IgG Ab）（＋）,EB（Epstein-Barr）病毒IgA抗体（EBV-CA-IgA Ab）（＋）。诊断为种痘水疱病样EB病毒感染相关T细胞增生性疾病。     

Erythema multiforme associated with cytomegalovirus infection in nonimmunosuppressed patients.

BACKGROUND: It is widely known that cytomegalovirus (CMV) primarily brings about subclinical and asymptomatic infection in the early stages of life and can cause various dermatological and systemic disorders under immunosuppressed conditions. Nonimmunosuppressed individuals very rarely present with cutaneous CMV involvement. OBJECTIVE: In the present study, we described the clinical characteristics of 5 nonimmunosuppressed adult patients with positive IgM antibody to CMV. METHODS: The systemic symptoms and dermatological features of these 5 patients were described. Laboratory examinations including blood cell counts, liver and renal functions were performed. IgG and IgM antibodies to CMV were also examined at the first consultation and 2-3 months after the skin eruption. Polymerase chain reaction for CMV DNA was performed in the skin samples of the patients. RESULTS: All 5 patients had fever and complained of a sore throat. Multiple exudative erythema and target lesions with itching were observed mainly on the extremities. These symptoms and eruptions disappeared within 1 week after the onset and IgM antibody titers significantly decreased after 2-3 months. IgG antibody to CMV was already positive in 3 cases but was negative in 2 cases at the initial consultation. CONCLUSION: We propose that CMV infection may cause erythema multiforme by primary, recurrent infections or reactivation of CMV even in nonimmunosuppressed adults.     

表现为嗜酸性粒细胞增多性皮炎的大疱性类天疱疮一例

患者,女,73岁。全身散在红斑水疱、弥漫性色素斑伴瘙痒1年。查体:躯干弥漫性条索状和漩涡状黑褐色色素沉着斑及散在绿豆至蚕豆大小不等的水疱,四肢有丘疹、结节样皮损,腹股沟淋巴结肿大。嗜酸粒细胞计数21.63×10~9/L,病理支持大疱性类天疱疮诊断。给予糖皮质激素、甲氨蝶呤同时联合干扰素α治疗后,病情得到明显缓解。     

Neonatal Lupus Erythematosus Occurring in Identical Twins

The patients were one-month-old identical twins. Scaly erythema was noted mainly on the trunk, face, and scalp of one twin starting about three weeks after birth and starting about two weeks after birth in the other. The patients' courses were observed without treatment; the eruptions tended to disappear two months after birth. The mother had a past history of transient facial erythema. Both twins and mother were positive for anti-nuclear antibody, anti-SS-A antibody, and anti-SS-B antibody. The histopathological findings corresponded to those of discoid lupus erythematosus (DLE). The class of HLA typing revealed Cw3 in both twins, which is frequently observed in neonatal lupus erythematosus (NLE), and A24 in the mother, which is frequently observed in mothers of babies with NLE.     

Long-term serological follow-up of patients treated for chronic cutaneous borreliosis or culture-positive erythema migrans

The kinetics of antibodies to Borrelia burgdorferi following successful treatment of early and late cutaneous borreliosis were analysed in consecutive serum samples by an enzyme-linked immunosorbent assay (ELISA) technique. Twenty-three patients with culture positive erythema migrans were followed for 23+/-14 months: 41% stayed seronegative, 35% showed an isolated immunoglobulin M (IgM) response, 8% an isolated IgG response and 16% a combined IgM and IgG responses. In general, antibody levels peaked within the first 3 months of symptom onset, whereafter a gradual decline was observed within 1 year. Twenty-two patients with chronic cutaneous borreliosis were followed for 23+/-11 months and all patients stayed IgG positive. Nearly three-quarters showed a clear decline in IgG levels over the years, while the rest did not. After 9+/-1 years 88% of 16 patients examined were still IgG positive. In conclusion, treatment of erythema migrans should be initiated on clinical appearance as a substantial number of patients stayed seronegative. Treatment success may in part be monitored serologically for both seropositive erythema migrans and chronic cutaneous borreliosis as most patients show declining titres after successful treatment. However, continuously high titres do not necessarily indicate treatment failure.     

成人斯蒂尔病1例

患者女,69岁。右侧面部红斑、水疱后疼痛14个月,反复发热、关节痛1年,全身散在分布皮疹5月。体检:面部片状暗红斑,上覆暗红色和黄色痂皮,痂皮下可见点状溃疡和脓性分泌物;背部及双侧上肢散在分布10余个约1.0cm×1.5cm大萎缩性斑块,表面有少许痂屑,部分挤压后有少许脓液;右侧肘部可见淡红色斑块,内侧有一直径约0.5cm的厚壁水疱,疱液稠。实验室检查:血白细胞、中性粒细胞均升高,血清铁蛋白增高,类风湿因子、抗核抗体阴性,抗生素治疗无效。皮损组织病理示:真皮浅层及血管壁部分淋巴细胞、中性粒细胞浸润,淋巴结活检呈反应性增生改变。诊断:成人斯蒂尔病。     

无水疱性类天疱疮一例

患者，男，70岁。全身泛发多形性红斑、丘疹伴瘙痒3个月，未见水疱。皮肤病理示真皮浅层血管周围轻度淋巴细胞、嗜酸粒细胞浸润。直接免疫荧光示表皮细胞间及基底膜IgG、C3、IgM、IgA阴性。间接免疫荧光示抗基底膜带IgG抗体1:40阳性。正常人皮肤盐裂间接免疫荧光示IgG表皮侧阳性。诊断为无水疱性类天疱疮，给予烟酰胺、四环素、雷公藤多苷口服，配合外用卤米松乳膏治疗，2个月后皮损基本消退。     

无水疱性类天疱疮二例并文献复习

报道2例无水疱性类天疱疮并复习以往相关文献。例1，男，72岁，因全身皮肤红斑、丘疹伴瘙痒20天就诊，查体未见水疱。实验室检查血嗜酸性粒细胞升高，直接免疫荧光示基底膜带线状C3沉积，间接免疫荧光示抗基底膜带抗体阳性，抗BP230抗体、抗BP180抗体阳性。例2，女，79岁。因全身皮肤散在分布丘疹伴严重瘙痒20天就诊，查体见躯干四肢皮肤抓痕明显，未见水疱。血清中抗BP180抗体阳性。2例均诊断：无水疱性类天疱疮。予以小剂量糖皮质激素治疗后，控制病情，目前在随访中。     

308nm准分子激光照射兔耳最小红斑量

目的:明确308 nm准分子激光照射兔耳最小红斑量(Minimum Erythema Dose,MED)。方法:应用308 nm准分子激光照射30只兔子左耳,分别给予单次单剂量150、300、450、600、900、1350mJ/cm~2,右侧作对照。计算30只兔耳激光照射24、48、72 h后的MED平均值,并观察96 h后组织病理和透射电子显微镜(transmission electron microscopy,TEM)下超微结构的改变。结果:经308 nm准分子激光照射后,24、48和72 h后MED平均值均为(300+55.39)mJ/cm~2,其中86.6%(24只兔)MED值为300 mJ/cm~2。照射96 h后MED区较未照射区的组织病理:表皮和真皮层增厚,真皮水肿、毛细血管扩张,血管周围大量炎症细胞浸润,可见红细胞外渗。TEM:MED区照射96 h后胶原纤维变性、排列紊乱,纤维细胞细胞器凝聚呈团块状变性;血管周围可见炎症细胞浸润,红细胞外渗;部分血管管腔狭窄,血管内皮细胞和周细胞细胞核形态异型。96 h后红斑逐渐消退,10~14天内消失,病理和TEM均未见大面积坏死。结论:308 nm准分子激光是一种高效稳定和安全的光源。     

Propylthiouracil-induced ANCA-positive erythema nodosum treated with thalidomide

Background Propylthiouracil (PTU), one of the mainstays of antithyroid therapy drugs, can lead to antineutrophil cytoplasmic antibody (ANCA) positivity and skin lesions. PTU‐induced ANCA‐positive vasculitis is rare and even more rare is erythema nodosum. Objective To report a case of a 57‐year‐old woman with hyperthyroidism who developed myeloperoxidase (MPO)‐ANCA erythema nodosum after PTU treatment for 11 months. Methods Skin biopsy demonstrated septal panniculitis without vasculitis. PTU‐induced ANCA‐positive erythema nodosum was made. Results With discontinuation of PTU and initiation of thalidomide, skin lesions resolved completely in three weeks, and after three months, the titers of MPO‐ANCA and perinuclear‐ANCA (p‐ANCA) had decreased remarkably. At 14‐month follow‐up, the patient was asymptomatic, but low levels of ANCA titers persisted. Conclusions This report indicated that ANCA positive erythema nodosum could develop following PTU treatment. Thalidomide has been proven to be helpful and averted the adverse effects from systemic corticosteroids and other immunosuppressive drugs in this patient.