Penicillamine-induced pemphigus foliaceus-like dermatosis. A case with unusual features, successfully treated by plasmapheresis

A case of a severe, widespread bullous dermatosis clinically resembling pemphigus foliaceus occurred during treatment with penicillamine hydrochloride in a patient with rheumatoid arthritis. Histologically, the disease showed changes compatible with pemphigus vulgaris as well as with a bullous drug eruption. Treatment by plasmapheresis proved to be effective in controlling the disease.     

环孢素治疗银屑病并发天疱疮一例

银屑病并发天疱疮临床少见,两种疾病治疗存在矛盾。该例患者有银屑病病史7年,20 d前出现水疱、糜烂;皮损组织病理及疱病自身抗体检查支持寻常性银屑病并发寻常性天疱疮诊断,给予口服环孢素及外用药物治疗后病情改善。     

Childhood pemphigus vulgaris successfully treated with rituximab

Pemphigus is a potentially fatal autoimmune epidermal bullous disorder. Rituximab is a novel therapy for the treatment of refractory pemphigus. However, there is limited clinical data on safety and efficacy of rituximab in pediatric age group. Herein, we report an 11-year-old boy of childhood pemphigus vulgaris who failed to respond to dexamethasone pulse therapy and was subsequently treated with rituximab and achieved complete remission.     

A case of lupus pneumonia treated by pulse therapies and plasma exchange therapy

A case of interstitial pneumonia accompanying SLE (lupus pneumonia) was reported. Shortness of breath in this patient was aggravating very rapidly even during methyl prednisolone pulse therapy, but the patient was relieved from this symptom soon after double filtration plasma exchange was carried out. We concluded that some cases which are resistant to pulse therapy and whose prognosis is very poor might be saved by plasma exchange.     

阿维A治愈以增殖性皮损为突出表现的家族性良性天疱疮一例

患者女,56岁,因外阴疣状赘生物1年余于2013年4月8日来我院就诊。患者无明显诱因于外阴及肛周出现菜花状赘生物,并迅速增大呈山峰状,易出血,表面破溃、糜烂、渗出伴异味,有瘙痒感觉。多家医院考虑巨大型尖锐湿疣予以抗病毒及物理治疗,疗效欠佳。追问病史,患者20年前无明显诱因乳房下皮肤出现红斑伴瘙痒,在当地医院治疗（具体方案不详）好转后反复发作,皮疹逐渐增多,扩展至双腋下及腰部并出现水疱、脓疱。5年前腰部再次出现上述皮疹,初起为红斑,迅速出现水疱,当地医院行分泌物培养：金黄色葡萄球菌,予对症治疗后好转出院（具体不详）。后因上述情况反复住院2次。2012年6月患者双侧乳房下、腋窝、腰腹部及腹股沟再次出现大片浸润性暗红斑片,其上见水疱、脓疱,部分破溃。既往有2型糖尿病史,否认肝炎、结核病病史,否认药物过敏史,家族中有类似患者。否认非婚性行为史,其丈夫多次检查无尖锐湿疣……     

A case of antiepiligrin cicatricial pemphigoid successfully treated by plasmapheresis

We report the case of a 73-year-old Japanese woman suffering from antiepiligrin (laminin 5) cicatricial pemphigoid (CP) with typical clinical and immunopathological features. Histologically, the lesional mucous membrane showed a subepidermal blister formation. When indirect immunofluorescence techniques with skin split by 1 M NaCl as the substrate were used, the patient's serum reacted only to the dermal side. Immunoprecipitation studies demonstrated that the patient's serum contained IgG autoantibodies directed against a set of polypeptides that corresponded to epiligrin (laminin 5). After corticosteroids and immunosuppressive agents had been administered systemically, the patient's autoantibody titer decreased and the cutaneous and mucosal blister formations were suppressed. However, the ocular lesions persisted in spite of these therapeutic regimens. After combining these treatments with double-filtration plasmapheresis, the ocular lesions improved and showed almost no progression. Plasmapheresis may thus present a new option for the treatment of CP.     

Angiolymphoid hyperplasia, a successfully treated case

Recurrent nodules on the face of a 37-year-old man, clinically and histologically diagnosed as angiolymphoid hyperplasia with eosinophilia, promptly disappeared on intralesional infiltration of hydroxypolyethoxydodecan, although his eosinophilia and elevated level of IgE continued.     

Controlled study of plasma exchange in pemphigus

To determine the potential steroid sparing effect of plasma exchange in pemphigus we enrolled 40 patients in a multicenter randomized study. Eighteen patients were treated by prednisolone alone, 22 by prednisolone plus ten large-volume plasma exchanges over four weeks. All patients received oral prednisolone in the same initial dosage (0.5 mg/kg/d), which was increased weekly if needed. The number of cases controlled at each therapeutic step did not differ between the two groups. In eight cases, four in each group, the disease was not controlled by the highest therapeutic step of the protocol, with four deaths from sepsis in the plasma exchange group. The controlled cases needed similar cumulative prednisolone doses (5237 +/- 5512 mg in the plasma exchange group vs 4246 +/- 1601 mg in the control group). The evolution of serum pemphigus antibody was not different in the two groups. These findings suggest that plasma exchange in association with low steroid doses alone are not effective in the treatment of pemphigus and may even promote sepsis.     

一例双膜血浆置换治疗寻常型天疱疮患者的护理体会

<正>天疱疮(pemphigus)是一种罕见且可危及生命的自身免疫性大疱性皮肤病,按照临床和病理特征,原发性天疱疮可分为寻常型、增殖型、落叶型和红斑型四种类型[1]。各型天疱疮皮肤黏膜损害的共同特点为:水疱、大疱发生在红斑或外观正常的皮肤上、疱壁松弛、尼氏征阳性[2]。疱壁易破形成糜烂面,糜烂面上附有淡黄色或褐色痂皮[3]。此外,口腔、咽喉、食管、外阴、肛门外生殖器黏膜常常受累,严重者可泛发全身[3-4],是自身免疫性大疱病中最常见且较为严重的皮肤病。     

血浆置换治疗一例难治性落叶型天疱疮伴感染的护理

<正>落叶型天疱疮是一种累及皮肤和黏膜的自身免疫性疱病,早期损害常限于头皮、面部和躯干上部,而后逐渐发展至全身,伴有大小不等的水疱,易破溃、有渗液、脱屑,伴恶臭[1]。临床上应用糖皮质激素及免疫抑制剂治疗,治疗难度大。血浆置换(plasma exchange)是根据血液中有形成分不同比重,通过离心分离血浆,进行置换,除去血浆中某些与发病有关的有害成分,从而减轻症状和缓解病情的方法 [2]。     

D-penicillamine-induced pemphigus successfully treated with combination therapy of mizoribine and prednisolone

A 63-year-old woman had D-penicillamine-induced pemphigus with a high index value of circulating autoantibodies against desmoglein 1. Because of the lack of response to prednisolone monotherapy, the patient was treated with a combination therapy of mizoribine and low-dose prednisolone. The skin eruption was improved by this combination with a declined value of circulating anti-desmoglein 1 antibody. Currently, a remission of 2 months has been achieved, and prednisolone could be tapered.     

A case of Rothmann-Makai panniculitis successfully treated with tetracycline

Rothmann-Makai syndrome (lipogranulomatosis subcutanea) is a rare variant of Weber-Christian disease, but lacks visceral involvements and systemic manifestations. We herein report the case of a 56-year-old Chinese woman with this disorder who complained of subcutaneous tender nodules over her extremities, trunk, and face of 2 years' duration. She was firstly treated with 300 mg of oral roxythromycin for 8 weeks and subsequently treated with 400 mg of oral clarithromycin for the next 8 weeks. However, no significant effect was observed. She was then administered 200 mg of oral minocycline hydrochloride. After 1 week, her skin symptoms rapidly improved and no relapse has been observed in a follow-up period of 3 months. Considering that tetracycline antibiotics, but not macrolide antibiotics, inhibit the activity of pancreatic lipase in vitro, the efficiency of tetracycline antibiotics may be a clue to clarifying the pathogenesis of this disorder.     

A case of rheumatoid nodulosis successfully treated with surgery

We report the case of a 76 year-old male with multiple subcutaneous nodules, but without joint symptoms or deformities, who was diagnosed histopathologically with rheumatoid nodulosis after resection of the nodules. Rheumatoid nodulosis is a disease characterized by multiple subcutaneous nodules that are histopathologically similar to rheumatoid nodules, a high titer of rheumatoid factor, and radiologically detectable cystic bone lesions, but with none or few of the systemic manifestations orjoint activity of rheumatoid arthritis (RA). It is considered to be a benign variant of RA. This rare disease must be considered when a case of multiple subcutaneous nodules is encountered, even in aged males withoutjoint symptoms or deformities.     

A case of erythrodermic psoriasis successfully treated with ixekizumab

Erythrodermic psoriasis (EP) is the most severe form of psoriasis, resulting in significant morbidity and mortality. International guidelines on EP treatment are lacking, with most of the biologic drugs being used basing on case reports or small case series. Ixekizumab, a fully human anti‐interleukin (IL)‐17A monoclonal antibody, is approved for moderate to severe plaque psoriasis while its use in EP is off label. However, two studies conducted on eight Japanese EP patients have showed ixekizumab as an efficacious and well tolerated therapy up to 24 and 52 weeks, respectively. To date, no case reports on Caucasian patients have been described. We report the case of a 66‐year‐old Caucasian female with EP successfully treated with ixekizumab, reaching PASI 100 after only 6 weeks of therapy and still maintaining this response at week 24. Our case report suggests ixekizumab as a highly efficacious treatment in EP, presenting also a very rapid action which leads to complete resolution of the disease after 6 weeks. Further studies are warrant to confirm our data, with controlled trials specifically dedicated to EP being strictly needed in order to verify the role and efficacy of the new biologics in EP.     

阿维A治愈甲银屑病一例

报告1例应用阿维A治愈的甲银屑病。患者女,52岁。10个指甲增厚、变色2月就诊。通过纵行甲活检获取的甲组织标本,病理学诊断为甲银屑病。阿维A30mg治疗8个月,10个指甲完全恢复正常厚度和颜色,随访7个月无复发。     

制霉菌素治愈原发性皮肤曲菌病一例报告

曲菌是一种很常见的腐物寄生性真菌,全世界各地都有,目前已发现这一属约包括300种左右的曲菌,大多腐生在土壤及腐烂植物上,但有些种如烟曲霉菌、黑曲菌及黄曲菌可致动物及人类发病.