Bullous systemic lupus erythematosus with antiphospholipid syndrome

We report the case of a 44-year-old male with a 10-year history of manifestations of the rare form of bullous systemic lupus erythematosus (SLE) with coexisting antiphospholipid syndrome (APS) that remained undiagnosed until thrombotic-embolic episodes appeared and high titres of anticardiolipin (ACL) antibodies were detected. The patient fulfilled the criteria for SLE and the atypical cutaneous manifestations together with histopathological changes and a favourable response to sulphones were the grounds for the diagnosis of the bullous variety of SLE. Treatment with prednisolone, acenocoumarol and dapsone resulted in marked clinical improvement, reduction in antinuclear antibodies (ANAs) and normalization of ACL antibody titres.     

药疹132例临床分析

目的 :探讨药疹与某些致敏药物的联系。方法 :查阅了 1998年 1月～ 1999年 12月我院药疹病历 132例 ,对致敏药物的种类及疹型进行了统计分析。结果 :怀疑致敏药物 6 5种 ,药疹的分型以荨麻疹型最多 ,其次为麻疹样型和猩红热型。结论 :引发药疹最多的药物是抗菌药物 ,其次是解热镇痛药和中药     

奥替溴铵致药疹1例

奥替溴铵 (ｏｔｉｌｏｎｉｕｍｂｒｏｍｉｄｅ)是一种选择性对消化道平滑肌有强烈解痉作用的化合物 ,它被用于平滑肌纤维细胞的病理性收缩亢进或痉挛病例。目前尚未发现奥替溴铵的任何具有临床意义的不良反应 ,所报道的不良反应均较轻微或与安慰剂相似 (恶心、疲乏、上腹痛和眩晕 ) ,在 138名用奥替溴铵进行治疗的肠易激综合征 (ＩＢＳ)病人中只有 2名用奥替溴铵和 1名用安慰剂的病人中断实验[1] 。奥替溴铵致药疹未见报道。笔者现遇到 1例报道如下。病人 ,男性 ,2 9ａ。因患肠易激综合征 ,服用奥替溴铵糖衣片 [商品名 :斯巴敏 (Ｓｐａｓｍ…     

口服阿卡波糖出现药疹1例

阿卡波糖(acarbose)为近年来临床上应用较广泛的降糖药，属α葡萄糖苷酶抑制剂，通过竞争抑制肠细胞刷状缘内的α葡萄糖苷酶，延迟蔗糖、糊糖、麦芽糖等多糖分解成单糖，并延迟葡萄糖在肠道的吸收，对餐后高血糖和高胰岛素血症效果较好。阿卡波糖的不良反应主要为肠胀气，皮肤损害较少见。我院应用阿卡波糖口服后出现双手大片药疹1例，现报道如下。     

Disorders characterized by predominant or exclusive dermal inflammation

Some cutaneous inflammatory disorders are typified by a predominant or exclusive localization in the dermis. They can be further subdivided by the principal cell types into lymphocytic, neutrophilic, and eosinophilic infiltrates, and mixtures of them are also seen in a proportion of cases. This review considers such conditions. Included among the lymphoid lesions are viral exanthems, pigmented purpuras, gyrate erythemas, polymorphous light eruption, lupus tumidus, and cutaneous lymphoid hyperplasia. Neutrophilic infiltrates are represented by infections, Sweet syndrome, pyoderma gangrenosum, and hidradenitis suppurativa, as well as a group of so-called “autoinflammatory” dermatitides comprising polymorphonuclear leukocytes. Eosinophil-dominated lesions include arthropod bite reactions, cutaneous parasitic infestations, the urticarial phase of bullous pemphigoid, Wells syndrome (eosinophilic cellulitis), hypereosinophilic syndrome, and Churg-Strauss disease. In other conditions, eosinophils are admixed with neutrophils in the corium, with or without small-vessel vasculitis. Exemplary disorders with those patterns include drug eruptions, chronic idiopathic urticaria, urticarial vasculitis, granuloma faciale, and Schnitzler syndrome (chronic urticarial with a monoclonal gammopathy).     

Acantholytic pityriasis rubra pilaris associated with topical use of imiquimod 5%: case report and literature review

Topical use of immune response modifiers, such as imiquimod, has increased in dermatology. Although its topical use is well tolerated, it may be associated with exacerbations of generalized cutaneous inflammatory diseases, possibly through the systemic circulation of pro-inflammatory cytokines. This report describes a case of development of pityriasis rubra pilaris, a rare erythematous-papulosquamous dermatosis, in a woman aged 60 years during treatment with imiquimod 5% cream for actinic keratosis. It evolved with erythrodermic conditions and palmoplantar keratoderma, presenting progressive clinical resolution after the introduction of methotrexate. The authors emphasize the importance of recognizing possible systemic reactions associated with the topical use of imiquimod.     

Efficacy of Granulocyte and Monocyte Adsorption Apheresis for Treatment of Palmoplantar Pustulosis

Palmoplantar pustulosis (PPP) is characterized by neutrophilic pustules with erythema, which are limited to the hands and feet. Although granulocyte and monocyte adsorption apheresis (GMA) has shown remarkable effects on generalized pustular psoriasis, there are few reports of PPP treated with GMA. We treated three refractory PPP patients using GMA weekly for 5 weeks. The skin eruptions were assessed by a 5‐grade score for scales, pustules, and erythema. GMA decreased the total grade from 9 to 2 in patients 1 and 2, and from 7 to 3 in patient 3. The GMA effects were estimated to be excellent in all three patients. Pustule formation and pain disappeared in all cases. The treatment effect lasted for at least 5 months after GMA. GMA was also effective for relieving the arthralgia in one patient, but it recurred at 6 weeks. Based on these findings, GMA could be an effective therapy for refractory PPP.     

A Case of Mucosal Fixed Drug Eruption Caused by Tamsulosin Administration

Fixed drug eruption (FDE) is a rare type of drug reaction that involves the skin and, less commonly, the mucosal membranes. It is characterized by clinically well-defined erythematous patches or plaques with or without blisters, which relapse at the same location if the causative agent is readministered. Tamsulosin is an alpha-1 adrenergic receptor blocker used to treat benign prostatic hyperplasia, and its common side effects are dizziness and headache. Only one case of cutaneous FDE due to tamsulosin administration has been reported but no other case of mucosal involvement has been reported to date. Therefore, we present a case of mucosal FDE caused by tamsulosin administration along with a literature review.     

A case of cutaneous extranodal natural killer/T-cell lymphoma clinically and histopathologically mimicking interface dermatitis

Extranodal natural killer/T-cell lymphoma (ENKTL) is a rare but aggressive cancer characterised by angiocentric and angiodestructive infiltration by NK-cells, or cytotoxic T-cell types. Histopathologically, ENKTL shows a multinodular or diffuse infiltration localised to vascular structures, resulting in angiodestruction and necrosis. We present a patient with an initially suspected diagnosis of benign interface dermatitis with a differential diagnosis of mycosis fungoides that was later found to be an aggressive extranodal natural killer/T-cell lymphoma of a nasal type and with a dismal prognosis.     

北京协和医院29 987例皮肤活检组织的临床病理诊断构成

【摘要】 目的 分析北京协和医院皮肤活检组织的疾病构成及临床和病理诊断情况。方法 回顾北京协和医院皮肤科2010年6月至2018年11月采用皮肤组织病理检查的29 987例患者,分析组织病理和诊断情况。结果 根据病理诊断结果,将确诊的皮肤疾病分为33大类,242种。常见的疾病类别分别为表皮肿瘤（2 931例,9.77%）、结缔组织病（2 809例,9.37%）、黑素细胞性肿瘤（2 078例,6.93%）、红斑鳞屑脓疱性皮肤病（1 376例,4.59%）、苔藓样皮肤病（1 291例,4.31%）、变态反应和湿疹类（1 282例,4.28%）及感染性疾病（1 156例,3.86%）等。常见疾病为硬皮病（1 887例,6.29%）、色素痣（1 755例,5.85%）、脂溢性角化（1 136例,3.79%）、湿疹（1 089例,3.63%）、银屑病（881例,2.94%）、扁平苔藓（867例,2.89%）、红斑狼疮（638例,2.13%）、天疱疮（549例,1.83%）和基底细胞癌（501例,1.67%）等。扁平苔藓、大疱性类天疱疮、环状肉芽肿、嗜酸性粒细胞增多性皮病等的临床诊断不符合率高,与病理诊断的一致率较低。结论 本院皮肤活检组织中表皮肿瘤、结缔组织病、黑素细胞性肿瘤、红斑鳞屑脓疱性皮肤病、苔藓样皮肤病、变态反应和湿疹类占比高。部分皮肤病临床诊断与病理诊断一致率低,对此类疾病需提高认识。