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。     

疱疹样天疱疮1例

患者，男，71岁。主诉：全身红斑水疱伴瘙痒2年，反复发作。现病史：患者2年前开始躯干、四肢陆续出现散在红斑、水疱，疱易破溃呈糜烂或结痂，瘙痒剧烈。中药治疗效果不佳，皮疹逐渐增多，来我科就诊。经病理检查诊断为红斑型天疱疮，给予强的松60mg qd，雷公藤多甙20mg tid口服，皮疹消退后强的松开始减量。减量期间皮损曾多次反复，近几次复发时躯干、四肢出现多数环形红斑，边缘有密集排列的小水疱，取腹部小水疱做病理检查示：棘层中部水疱，疱内可见多数中性和少量嗜酸粒细胞浸润，真皮浅中层血管周围有淋巴细胞、组织细胞及嗜酸粒细胞浸润（图1）。直接免疫荧光检查（DIF）未发现表皮细胞问IgG、IgA、     

胫前大疱性表皮松解症并发落叶型天疱疮1例

报告胫前大疱性表皮松解症并发落叶型天疱疮1例。患者男,64岁。全身红斑、瘙痒1年,出现松弛性水疱、糜烂、结痂5个月。患者自10岁始四肢出现瘙痒性疱疹,渐形成红色瘢痕,并持续至今。皮损组织病理检查：大疱处示棘层上部大疱,疱中有嗜酸性纤维蛋白网、大量中性粒细胞及棘层松解细胞;胫前处皮损示表皮下裂隙性水疱,较多成纤维细胞和结缔组织。间接免疫荧光检查抗表皮细胞间基质抗体（＋）,滴度为1：40。     

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。     

线状IgA大疱性皮病1例

患者男,45岁.因全身泛发水肿性红斑、水疱2个月,于2008年4月收入院.患者2个月前无明显诱因全身出现散在红斑、水疱.大小不一,自觉剧烈瘙痒,口腔深部黏膜糜烂、疼痛,在当地医院诊断为"天疱疮",给予口服地塞米松15 mg/d及对症支持治疗后症状好转,皮损逐渐消退,遗留暗紫色瘢痕.     

发热性溃疡坏死性急性痘疮样苔藓样糠疹1例

患者男,26岁,全身出现红斑、溃疡及结痂1个月,伴发热1 d。躯干四肢及身体屈侧可见广泛分布的大小不一的疼痛性红斑,部分红斑表面可见小水疱、溃疡和黑色浆痂。皮损组织病理检查示:角化过度,表皮增生,部分表皮坏死,基底细胞空泡化,表皮内可见散在的坏死角质形成细胞,表皮下水疱形成,真皮乳头及浅层血管周围可见不等量淋巴细胞浸润。诊断:发热性溃疡坏死性急性痘疮样苔藓样糠疹。     

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。     

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.     

重症大疱性类天疱疮2例的治疗体会

最近笔者采用大剂量皮质类固醇冲击治疗了2例大疱性类天疱疮患者,取得明显疗效,现报告如下。例1.男,65岁。以四肢红斑、水疱伴瘙痒40d于2002年月4日入院。患者于入院前40d无明显原因双前臂屈侧手腕附近出现十几个黄豆大的水疱,基底红,疱液透明,疱壁紧张伴有轻度瘙痒。2d后疱壁部分破溃,很快干燥结痂,以后又不断出现新发水疱。发疹范围渐波及颈部、上胸部、背部及双下肢,水疱此起彼伏,可自行结痂愈合。皮损组织病理检查示:表皮增生肥厚,细胞内水肿,可见表皮下水疱形成;直接免疫病理:基底膜带可见IgG、C3沉积,IgA(-)。遂给予泼尼松40mgd、雷…     

泛发性家族性良性天疱疮

报告1例泛发性家族性良性天疱疮.患者男,56岁.反复发作全身瘙痒性皮损40余年,皮损表现为以躯干部位为主的泛发枉绿豆到黄豆大红斑或斑丘疹,以及在红斑或正常皮肤上出现的水疱.皮损组织病理检查示表皮内广泛的棘层松解,并形成表皮内水疱.直接免疫荧光检查结果阴性.家系调查示该家族66人中共有12人患此病.诊断:泛发性家族性良性天疱疮.机械摩擦、感染和搔抓是该病加重的重要因素.     

Erythema gyratum repens responding to cetirizine hydrochloride

We report a case of erythema gyratum repens (EGR) in a 59-year-old man with inoperable pancreatic cancer and liver metastasis. The patient had a widespread erythema with concentric marginal band spreading in waves over the trunk and extremities. Numerous vesicles were seen on the margin of the erythema. The skin lesions were severely pruritic, and his peripheral blood showed marked eosinophilia. He also had palmoplantar hyperkeratosis. A biopsy specimen of the erythema disclosed spongiosis, microvesicles filled with eosinophils, infiltration of eosinophils into the epidermis, and a perivascular infiltrate in the dermis. The skin lesions and pruritus cleared quickly after the administration of cetirizine hydrochloride.     

柳氮磺胺吡啶致重症药疹1例

患者男,57岁。全身皮肤红斑、瘀斑伴瘙痒、发热10d。患者1个月前因"溃疡性结肠炎"口服"柳氮磺胺吡啶",20d后全身皮肤出现红斑、瘀斑伴瘙痒、发热。血常规检查示单核细胞及嗜酸性粒细胞增多,血小板减少;肝功能异常;胸部CT平扫示腋窝淋巴结肿大。结合病史、临床表现和辅助检查诊断为:重症药疹。予甲基泼尼松龙联合人免疫球蛋白等治疗痊愈。     

扁平苔藓样药疹1例

患者男,77岁。全身出现红斑和丘疹,伴瘙痒7天。2个月前,患者口服"厄贝沙坦、美托洛尔、苯磺酸氨氯地平片、阿托伐他汀钙、阿司匹林、硫酸氯吡格雷、埃索美拉唑镁肠溶片"等药物。7天前,全身出现散在分布的粟粒至蚕豆大红斑和丘疹,以躯干和四肢近心端为著,瘙痒剧烈。既往无药物过敏史。左小腿胫前新鲜水疱组织病理示:表皮角化过度,棘层肥厚,基底细胞空泡样变性,真皮浅层以淋巴细胞为主的带状浸润,可见嗜酸性粒细胞及胶样小体。诊断:扁平苔藓样药疹。     

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。     

Adult linear IgA bullous dermatosis: a polymorphic disorder

We report on two patients with unusual forms of adult linear IgA bullous dermatosis. One was a middle-aged woman who had targetoid lesions and bullae on her trunk and extremities. This patient first presented with lesions that clinically resembled erythema multiforme, but these evolved into a widespread eruption with bulging, elongated bullae. Examination of a biopsy specimen showed changes compatible with dermatitis herpetiformis and bullous pemphigoid. Findings on immunofluorescence studies showed deposition of linear IgA at the basement membrane zone. The second patient was an elderly woman with intensely pruritic vesicles whom we classified as having vesicular pemphigoid, until the linear IgA band on direct immunofluorescent test results became the predominant immunofluorescent finding. These cases are reported because of their unusual clinical presentations. The mechanism for the targetoid lesions in the first patient is discussed.     

The time course of the change in antibody titres in herpes gestationis

The time course of the change in antibody titres was examined postpartum after treatment in two patients with herpes gestationis. The first patient, a 29-year-old woman seen first in the 32nd week of her first pregnancy, had an exudative erythema, and developed an itchy erythema with small tense vesicles on the trunk and legs after delivery in the 40th week of pregnancy. The second patient, a 28-year-old woman seen first in the 28th week of her first pregnancy, had an itchy exudative erythema, small tense vesicles and crusts on the legs. After a Caesarean section in the 40th week of pregnancy performed because of cardiac complications in the fetus, the skin lesions extended to the trunk and extremities. Direct immunofluorescence revealed linear depositions of IgG and C3 at the basement membrane zone (BMZ) and indirect immunofluorescence was positive at the epidermal side of the BMZ in 1 mol/L NaCl-split skin in both cases. In patient 1, prednisolone, 20 mg/day, administered 4 months after delivery, gave rapid improvement (within 1 week) of the skin lesions; in patient 2, minocycline, 200 mg/day, administered 2 weeks after delivery, gave improvement within 2 weeks. Immunoblotting against epidermal extracts revealed the presence of antibodies directed to the 180 kDa bullous pemphigoid antigen in both sera. Indirect immunofluorescence and immunoblot were positive for at least 2 months in patient 1 and for 5 months in patient 2 after disappearance of the skin lesions.     

Dermatitis herpetiformis presenting as chronic urticaria

Childhood dermatitis herpetiformis (DH) is an immunobullous disease associated with gluten-sensitive enteropathy. This disease is rare in children and is typically characterized by intensely pruritic vesicles on the extensor surfaces. Definitive diagnosis of DH depends on the direct immunofluorescence finding of granular or fibrillar IgA deposits along the basement membrane zone of biopsied perilesional skin. We report an 11-year-old boy with an unusual presentation of DH characterized by a 7-month history of chronic urticaria-like skin lesions. He had evanescent, largely asymptomatic, urticarial wheals on his trunk, face, and extremities that were unresponsive to conventional therapy for urticaria. Skin biopsy specimen findings were consistent with DH and direct immunofluorescence of perilesional skin was diagnostic. The patient had no symptoms of gluten-sensitive enteropathy at the time of diagnosis, and his skin lesions rapidly cleared with dapsone therapy. This patient serves to highlight an unusual presentation of childhood DH and the need to consider this diagnosis when evaluating chronic urticarial lesions in children.     

Erythema nodosum induced by the synergism of acupuncture therapy and flu-like infection

A 32-year-old female patient developed erythema nodosum-like lesions at needle prick sites after acupuncture therapy. Over the next few days, she developed similar new lesions over the extremities, trunk and face along with flu-like symptoms. There were neither genital ulcerations nor eye lesions. A skin biopsy specimen from an extremity lesion showed the characteristic findings of erythema nodosum. Treatment with oral potassium iodide at a dose of 750 mg/day was effective, and there has not been any recurrence to date. We diagnosed this case as erythema nodosum induced by a synergism between acupuncture therapy and a flu-like infection.     

Two cases of autoimmune progesterone dermatitis. Immunohistochemical and serological studies

Two cases of autoimmune progesterone dermatitis are reported. The patients developed recurrent pruritic erythematous and edematous eruptions on the extremities, trunk or face, with occasional vesicles on the palms and soles. The eruptions appeared 7 to 10 days prior to their menstruation and persisted for several days. They showed immediately positive skin tests with 0.1 mg/ml and 0.2 mg/ml of aqueous progesterone suspension, respectively. The patients had IgG serum factor which bound rat corpus luteum. Positive indirect basophil degranulation tests against progesterone were demonstrated in both patients. Circulating autoantibodies to patients' own progesterone may cause or modulate the intermittent eruptions of the disease.     

CHRONIC BULLOUS DERMATOSIS OF CHILDHOOD: DIFFERENTIATION FROM OTHER AUTOIMMUNE BLISTERING DISEASES IN CHILDREN

An 8-year-old girl presented with a 1-week history of intensely pruritic vesicles and bullae on the trunk and extremities. The patient was otherwise in good health and was not taking any medications.
Physical examination revealed multiple large, tense bullae on normal-appearing skin on the trunk, extremities, and pelvic region. Some of the bullae were hemorrhagic, while others were grouped in circular clusters (Fig. 1A, B). The oral cavity and conjunctivae were spared. Complete blood cell count, electrolytes, serum urea nitrogen, creatinine, and liver function studies were normal.
Histopathologic examination of perilesional skin demonstrated a subepidermal blister with neutrophils in the dermal papillae (Fig. 2). Direct immunofluorescence of the specimen showed heavy deposition of IgA in a linear pattern in the basement membrane zone (Fig. 3). Broad but faint deposits of IgM were also present. Blood obtained for indirect immunofluorescence revealed no circulating autoantibodies.
The patient was diagnosed with chronic bullous dermatosis of childhood and treated with dapsone (2 mg/kg) with dramatic improvement. New blister formation and puritis were suppressed within several days. All lesions healed with mild hyperpigmentation but without scarring.