A case of bullous pemphigoid induced by tiobutarit (D-penicillamine analogue)

A 61-year-old woman with severe rheumatoid arthritis developed bullous lesions on the trunk, conjunctiva, and oral mucosa during treatment with tiobutarit, a new antirheumatic drug analogus to D-penicillamine. Histology showed a subepidermal blister, and circulating antibodies to the basement membrane zone were detected. The bullous lesions improved rapidly after discontinuation of tiobutarit. However, 3 years later, she was re-exposed to tiobutarit and the bullous lesions recurred within a week. This is the first confirmed case of tiobutarit-induced BP.     

高龄心脑血管疾病并发大疱性类天疱疮1例护理

目的：进一步加强对大疱性类天疱疮的认识,探讨合理的护理方案在高龄心脑血管疾病并发大疱性类天疱疮治疗中的重要性。方法：总结对高龄心脑血管疾病并发大疱性类天疱疮患者的护理措施及护理经验。结果：回顾病例,严密观察病情,治疗上予以口服及外用激素,护理上予以生活护理、饮食护理、严格消毒隔离、加强皮肤黏膜护理,采用改良疱液抽取术联合擦药护理,同时结合药物观察、心理支持和健康教育等,促进创面愈合,减少继发感染,促进疾病痊愈。结论：对于高龄心脑血管疾病并发大疱性类天疱疮患者,应早诊断、早治疗,联合专业护理,预防并发症,提高生活质量。     

Combination chemotherapy in a patient with severe multiple systemic autoimmune disease

We describe the occurrence of autoimmune haemolytic anaemia (AIHA) in a patient who had had both idiopathic thrombocytopenic purpura (ITP) and bullous pemphigoid (BP) for about 10 years. All of these diseases worsened rapidly, and the patient's clinical condition became critical after an episode of acute pneumonia. His AIHA, ITP and BP were responsive to combination chemotherapy consisting of cyclophosphamide, vincristine and prednisolone (CVP therapy).     

Therapy of autoimmune mouth bullous disease: 2020 review

Most diseases of oral mucosa are either autoimmune in nature or are the results of immunologically mediated events. The diseases with autoimmune pathogenesis are namely pemphigus and pemphigoid; the oral involvement is frequent or regularly observed in these diseases. The treatments with traditional drugs or biologic agents or combinations of these molecules are employed in clinical practice. New therapeutic targets aim to provide new treatment strategies that may go beyond nonspecific immunosuppression.     

Acquired Hemophilia A associated with bullous pemphigoid: A rare combination

Acquired Hemophilia A (AHA) is a rare autoimmune disorder, caused by the development of circulating autoantibodies against coagulation factor VIII (FVIII). AHA is associated with bullous pemphigoid in 2% of patients. We report a case of a 74-year-old man admitted with anemia and a tense subcutaneous and muscular hematoma in the right thigh. Blood analysis confirmed AHA. The patient had a recent diagnosis of bullous pemphigoid. Response to bypass agents and corticosteroids was good with bleeding control and normalization of FVIII and negative autoantibodies, respectively. In a 3-month follow-up period after tapering and stopping prednisolone, a relapse occurred, and immunosuppression was reinitiated. An early diagnosis and effective treatment in AHA are essential to reduce morbimortality. A careful tapering of immunosuppression is important to minimize FVIII inhibitor recurrence, as observed in this case.     

六例结节性类天疱疮临床和实验室检查特点

目的探讨6例结节性类天疱疮临床表现和实验室检查特征。方法回顾性分析2016年1月至2019年8月北京协和医院皮肤科住院部6例结节性类天疱疮患者临床资料。结果结节性类天疱疮主要发生于中老年人,平均发病年龄(58±16)岁。4例嗜酸性粒细胞升高,5例进行了免疫球蛋白E(IgE)检测,均升高,平均(530±672) KU/L。直接免疫荧光均提示基底膜带IgG和/或C3沉积,间接免疫荧光示抗基底膜带IgG抗体阳性,滴度1∶40～1∶320。5例患者抗BP180阳性[(24～85)U/ml,平均(43±26) U/ml],未合并中枢神经系统疾病。6例患者中1例失访,5例可随访的患者中3例出现复发,2例仍口服激素维持治疗。结论结节性类天疱疮发病年龄相对较轻,嗜酸性粒细胞和总IgE升高,抗BP180抗体滴度低,容易复发但合并神经系统疾病较少。     

Activation of blood coagulation in bullous pemphigoid: role of eosinophils, and local and systemic implications

Background Bullous pemphigoid (BP) is a blistering skin disease caused by autoantibodies to hemidesmosomal proteins, with eosinophils participating in blister formation. Eosinophils are a source of tissue factor (TF), an initiator of blood coagulation. Objectives To evaluate the local and systemic activation of coagulation in BP. Methods We studied 20 patients with active BP (eight re‐evaluated during remission) and 40 controls. The coagulation markers prothrombin fragment F1+2 and d ‐dimer were measured in the plasma of all subjects and in both plasma and blister fluid of patients with BP. TF was evaluated immunohistochemically in skin specimens from the 20 patients and in 20 normal samples. Results F1+2 and d ‐dimer levels were higher in plasma of patients with BP (649 ± 96 pmol L^?1 and 18·52 ± 3·44 nmol L^?1, respectively) than in plasma of controls (157 ± 7 pmol L^?1 and 1·42 ± 0·06 nmol L^?1; P = 0·0001), and were very high in blister fluid (40 449 ± 3491 pmol L^?1 and 1532·32 ± 262·81 nmol L^?1; P = 0·0001). Plasma and blister fluid F1+2 and d ‐dimer levels paralleled blood and tissue eosinophilia and disease severity. In the eight patients re‐evaluated during remission, there was a marked reduction in F1+2 (from 1127 ± 144 to 287 ± 52 pmol L^?1; P = 0·005) and d ‐dimer (from 24·03 ± 4·08 to 4·69 ± 1·51 nmol L^?1; P = 0·029). Immunohistochemistry revealed strong TF reactivity in BP skin (P = 0·0001), and colocalization studies confirmed eosinophils as a source of TF. Conclusions The coagulation cascade is activated in BP and correlates with the severity of the disease and with eosinophilia, indicating that eosinophils play a role in coagulation activation via TF. The hypercoagulability may contribute to inflammation, tissue damage, blister formation and possibly thrombotic risk in BP.