大疱性色素性荨麻疹1例

患儿女,4个半月。全身色素斑4个月,起水疱10天。查体:全身弥漫性灰褐色色素斑,表面见较多水疱、大疱及糜烂结痂,尼氏征阴性,Darier征阳性。皮损组织病理检查显示真皮浅层及血管周围见较多单一核细胞浸润,G iem sa染色阳性。诊断为大疱性色素性荨麻疹。     

A Case of Bullous Dermatitis Induced by Erlotinib

Herein, we report a case of bullous dermatitis that occurred in a 61‐year‐old woman 5 days after beginning therapy with erlotinib for the treatment of stage IV pulmonary adenocarcinoma with metastases at the hypophyseal level. Skin reactions are the most common adverse drug reactions (ADRs) associated with epidermal growth factor receptor tyrosine kinase (EGFR‐TK) inhibitors, and acneiform rash is the most frequently reported ADR in patients treated with erlotinib. To our knowledge, this is the first case of bullous dermatitis induced by erlotinib. This report highlights the need for additional research in the field of skin toxicity of EGFR‐TK inhibitors.     

Increased serum IL-6, TNF-alpha and IL-10 levels in patients with bullous pemphigoid: relationships with disease activity

AIM: The present report analyzes the serum levels of three cytokines, interleukin-6 (IL-6), tumor necrosis factor-alpha (TNF-alpha) and interleukin-10 (IL-10) in 15 patients with bullous pemphigoid (BP) (compared with 20 healthy controls) to evaluate a possible involvement of these biological modulators in the clinical expression of this disease. BACKGROUND: BP is a rare bullous disease of autoimmune origin with evidence of inflammatory processes that cause skin lesions with local increase of various pro-inflammatory mediators. METHODS: Determination of cytokine concentrations were obtained employing commercially available ELISA kits. RESULTS: The sera of BP patients showed increased levels of these three cytokines (P < 0.01). When the number of skin lesions (blisters and/or erosion) of each patient, employed as a marker of disease activity, was correlated with the serum levels of IL-6 and TNF-alpha, significant correlations were found (IL-6: P < 0.01 and TNF-alpha: P < 0.01, respectively), suggesting a possible role of these mediators in the development of BP blisters. The serum levels of IL-6 also correlated (P = 0.01 with those of serum C reactive protein (CRP), an acute-phase protein induced by IL-6 in hepatocytes. In addition, serum TNF-alpha and sE-selectin (an adhesion molecule previously reported to be increased by this cytokine) levels were also correlated (P < 0.05). CONCLUSIONS: On the basis of these data, it may be indicated that at least IL-6 and TNF-alpha are associated with the clinical expression of BP and that the endothelial activation (possibly induced by the TNF-alpha activity), seems to be an important phase of this dermatosis.     

Burn-induced linear IgA dermatosis

There have been several reports of linear IgA dermatosis (LAD) associated with drug exposure and lymphoproliferative malignancy, but trauma and burns have been suggested only in patients with bullous pemphigoid. We present a case of burn-induced LAD in a 48-year-old caucasian male presenting with a recent history of blistering eruption on the periphery of a cicatricial area caused by boiling methyl alcohol. Clinically, he presented a widespread bullous eruption. The direct immunofluorescence examination of a perilesional biopsy revealed an intense homogeneous linear pattern of IgA deposition consistent with the diagnosis of LAD. The patient responded to therapy with systemic steroids.     

A population-based study of acute medical conditions associated with bullous pemphigoid

Background  Bullous pemphigoid is associated with poorly understood dramatically increased early mortality rates.
Objectives  To assess the incidence of acute events predisposing to early mortality.
Methods  Computerized medical records from the Health Improvement Network, a large population-based U.K. general practice database, were used to conduct a cohort analysis. Outcome measures were incidence rates of myocardial infarction, pulmonary embolism, pneumonia and sepsis compared with a matched control population.
Results  People with bullous pemphigoid were three times as likely to develop pneumonia, adjusted rate ratio 2·94 [95% confidence interval (CI) 2·01–4·31] or pulmonary embolism, adjusted rate ratio 3·12 (95% CI 1·37–7·12) compared with matched controls. No statistically significant increase was seen for myocardial infarction, adjusted rate ratio 1·24 (95% CI 0·66–2·33), or sepsis, adjusted rate ratio 2·02 (95% CI 0·78–5·21).
Conclusions  The risk of pulmonary embolism and pneumonia is increased following a diagnosis of bullous pemphigoid. It may be possible to reduce associated mortality through considering prophylaxis with either antithromboembolic measures or antibiotic therapy and vaccination.     

经皮穿刺肺大疱内固定减容术治疗巨大肺大疱

目的观察肺大疱内固定减容术治疗肺大疱的疗效。方法采取前瞻性自身对照的方法，对22例（个）巨大肺大疱实施肺大疱内固定减容术。结果22例肺大疱中最小者约10 cm×7 cm×5 cm（前后径×内外径×上下径，下同），最大者约15 cm×8 cm×30 cm（累及一侧整半个胸腔）。19例在术后7天内肺大疱闭合，1例在术后15天闭合，2例在术后10天和14天缩小并稳定，所有病例均获呼吸困难减轻或消失，肺功能改善。随访4-42个月，失访2例，余无1例复发和加重。结论CT引导经皮肺大疱内固定减容术只需局麻，创伤小、费用低、并发症少、疗效确切，是巨大肺大疱的安全有效的治疗方法。     

电视胸腔镜手术治疗巨大肺大疱

目的 :探讨电视胸腔镜手术治疗巨大肺大疱的可行性和效果。方法 :对 1 4例 (1 5侧肺叶 )临床资料进行回顾性分析。患者肺大疱均占据胸腔 >50 % ,术前呼吸困难 4级 7例 ,5级 7例。结果 :本组 1 4例病人 1 5侧肺叶 ,其中 2侧肺叶在单纯胸腔镜下完成大泡切除 ,1 3侧肺叶在小切口辅助下施行了肺大泡切除。手术过程顺利。手术时间 90～ 2 4 0min ,出血 50～ 80 0ml。术后呼吸困难情况明显改善 :指标提升 1级 8例 ,提升 2级 6例 (如 5级提升至 3级 )。术后主要并发症为术后漏气。结论 :电视胸腔镜手术治疗巨大肺疱是可行和安全的 ,切割方法和壁层胸膜摩擦固定术是防止复发的首选方法     

Coagulation activation in autoimmune bullous diseases

The main autoimmune blistering skin disorders are pemphigus vulgaris (PV) and bullous pemphigoid (BP). They differ in the inflammatory infiltrate, which is more intense in BP. Inflammation is known to activate coagulation in several disorders. Local and systemic activation of coagulation was evaluated in BP and PV. We studied 20 BP patients (10 active and 10 remittent), 23 PV patients (13 active and 10 remittent) and 10 healthy subjects. The coagulation markers prothrombin fragment F1+2 and D‐dimer were measured by enzyme‐immunoassays in plasma. The presence of tissue factor (TF), the main initiator of blood coagulation, was evaluated immunohistochemically in skin specimens from 10 patients with active PV, 10 patients with active BP and 10 controls. Plasma F1+2 and D‐dimer levels were significantly high in active BP (P = 0·001), whereas in active PV the levels were normal. During remission, F1+2 and D‐dimer plasma levels were normal in both BP and PV. TF immunoreactivity was found in active BP but neither in active PV nor in normal skin. TF reactivity scores were higher in active BP than in controls or active PV (P = 0·0001). No difference in TF scores was found between active PV and controls. BP is associated with coagulation activation, which is lacking in PV. This suggests that BP but not PV patients have an increased thrombotic risk. The observation that thrombotic complications occur more frequently in BP than in PV further supports this view.     

幼儿大疱性类天疱疮1例

报道1例儿童大疱性类天疱疮。患儿男．1岁8个月。全身泛发大疱生皮损，疱壁紧张，不易破裂，疱液清澈，尼氏征阴性。皮肤组织病理检查见表皮下水疱，真皮层见以嗜酸粒细胞为主的浸润。皮肤免疫病理检查见表皮基膜带有IgM及C3沉积，患儿经皮质类固醇治疗，疗效满意。