bullous disorders

大疱性疾病
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  • 文章类型: Case Reports
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  • 文章类型: Journal Article
    未经证实:泡状甲虫皮炎(BBD)和带状疱疹(HZ)突然出现,水泡性病变相互模仿,进展迅速。但是,由于缺乏明确的区分标准,因此需要更好的调查方式。尽管组织病理学说服了这种需要,是一种侵入性程序,通常被推迟。因此,皮肤镜,一种非侵入性快速诊断工具,可以帮助区分。
    UNASSIGNED:评估BBD和HZ的不同皮肤镜模式,以区分两者,并研究病变早期和晚期的皮肤镜特征。
    UNASSIGNED:在印度南部进行的观察性横断面研究。招募了9例具有提示BBD和HZ的临床特征的患者。病变被任意分为早期和晚期。用手持式皮肤镜进行皮肤镜检查。必要时通过皮肤活检和Tzanck涂片证实诊断。使用频率和百分比方面的数据进行统计分析。
    UNASSIGNED:早期BBD病变的皮肤镜检查显示多个离散和融合的黄白色结构,棕色圆点,圆形白色小球,灰色结构,\'targetoid模式\',棕色区域在强烈的带红色的粉红色背景上。晚期BBD病变显示粉红色白色区域,减少灰色结构,点状和球状血管。早期HZ病变显示多小叶灰色和棕色小球,明亮的粉红色背景,被灰色面纱状结构覆盖的灰色小球具有灰色边缘。HZ晚期病变显示出“日食”模式和“皱巴巴的织物”模式。皮肤镜检查结果与组织病理学相关。
    未经评估:皮肤观察模式显示出与BBD和HZ一致的独特特征,因此,有助于早期诊断,协助在这两种情况下的准确治疗。
    UNASSIGNED: Blister beetle dermatitis (BBD) and herpes zoster (HZ) manifest suddenly with vesicular lesions mimicking each other and progress rapidly. But a lack of definite differentiating criteria yearns the need for better investigating modality. Though histopathology persuades the need, is an invasive procedure, commonly deferred. Thus, dermoscopy, a non-invasive rapid diagnostic tool, can help in differentiating.
    UNASSIGNED: To evaluate different dermoscopic patterns of BBD and HZ to differentiate both and to study dermoscopic features in early and late stages of lesions.
    UNASSIGNED: An observational cross-section study conducted in southern India. Nine patients with clinical features suggestive of BBD and HZ were recruited. Lesions were divided arbitrarily into early and late. Dermoscopic examination was performed with handheld dermoscope. Diagnosis was confirmed by skin biopsy and Tzanck smear wherever necessary. Statistical analysis performed using data in terms of frequencies and percentages.
    UNASSIGNED: Dermoscopy of early BBD lesions showed multiple discrete and confluent yellowish-white structures, brown dots, roundish white globules, gray structures, \'targetoid pattern\', brown areas over intense reddish pink background. Late BBD lesions revealed pinkish-white area, reduced gray structures and, dotted and globular vessels. Early HZ lesions showed poly-lobular gray and brown globules, bright pink background, gray globules covered by grayish veil-like structure with gray rim. Late HZ lesions revealed \'solar eclipse\' pattern and \'crumpled fabric\' patterns. The dermoscopic findings correlated with histopathology.
    UNASSIGNED: Dermoscopic patterns show peculiar features consistently pertaining to BBD and HZ, thus help in early diagnosis assisting in accurate treatment in both conditions.
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  • 文章类型: Journal Article
    Bullous pemphigoid (BP) is an autoimmune blistering skin disease that takes a profound physical and mental toll on those affected. The aim of the study was to investigate the bidirectional association between BP and all bullous disorders (ABD) with a broad array of psychiatric disorders, exploring the influence of prescribed medications.
    This nationwide, register-based cohort study encompassed 6,470,450 individuals born in Denmark and alive from 1994 to 2016. The hazard ratios (HRs) of a subsequent psychiatric disorder in patients with BP/ABD and the reverse exposure and outcome were evaluated.
    Several psychiatric disorders were associated with increased risk of subsequent BP (4.18-fold for intellectual disorders, 2.32-fold for substance use disorders, 2.01-fold for schizophrenia and personality disorders, 1.92-1.85-1.49-fold increased risk for organic disorders, neurotic and mood disorders), independent of psychiatric medications. The association between BP and subsequent psychiatric disorders was not significant after adjusting for BP medications, except for organic disorders (HR 1.27, CI 1.04-1.54). Similar results emerged with ABD.
    Psychiatric disorders increase the risk of a subsequent diagnosis of BP/ABD independent of medications, whereas medications used for the treatment of BP/ABD appear to account for the subsequent onset of psychiatric disorders. Clinically, an integrated approach attending to both dermatological and psychiatric symptoms is recommended, and dermatologists should remain vigilant for early symptoms of psychiatric disorders to decrease mental health comorbidity.
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  • 文章类型: Journal Article
    BACKGROUND: Bullous pemphigoid (BP) is an autoimmune blistering disorder occurring mostly in the elderly that lacks adequate treatments.
    OBJECTIVE: To describe our experience using dupilumab in a series of patients with BP.
    METHODS: This is a case series of patients from 5 academic centers receiving dupilumab for BP. Patients were eligible if they had a clinical diagnosis of BP confirmed by lesional skin biopsy evaluated by one of more of the following: hematoxylin and eosin staining, direct immunofluorescence, or enzyme-linked immunosorbent assay for BP180 or BP230, or both.
    RESULTS: We identified 13 patients. Patients were an average age of 76.8 years, and the average duration of BP before dupilumab initiation was 28.8 months (range, 1-60 months). Disease clearance or satisfactory response was achieved in 92.3% (12 of 13) of the patients. Satisfactory response was defined as clinician documentation of disease improvement and patient desire to stay on the medication without documentation of disease clearance. Total clearance of the BP was achieved in 53.8% (7of 13) of patients No adverse events were reported.
    CONCLUSIONS: Include small sample size, lack of a control group, lack of a standardized assessment tool, and lack of standardized safety monitoring.
    CONCLUSIONS: Dupilumab may be an additional treatment for BP, leading to disease clearance or satisfactory response in 92.3% of patients, including in those in whom previous conventional therapy had failed.
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  • 文章类型: Case Reports
    Background: Pemphigus vulgaris is an autoimmune blistering disease affecting the skin and mucous membranes. Current treatments for pemphigus vulgaris include anti-inflammatory and immunosuppressive agents. Rituximab, an anti-CD20 monoclonal antibody, has been shown to be effective for the treatment of pemphigus vulgaris. However, the optimal dosage of rituximab for the treatment of this autoimmune bullous disease has not been clearly defined.The aim of this study was to investigate the clinical efficacy and adverse effects of an ultra-low dosage regimen of rituximab for pemphigus vulgaris.Methods: We performed a prospective non-randomized open case series including eight patients affected by pemphigus vulgaris. Patients were treated with an ultra-low dosage of rituximab (a single infusion of 200 mg).Results: All patients had a positive response after infusion. At the end of the follow-up period, 5 patients achieved a complete remission and 3 a partial remission. Except for one case of sepsis due to Citrobacer freundii and a pneumonia due to Haemophilus influenzae, no adverse events were documented in our patients.Conclusions: Data from our study suggest that an ultra-low dosage of rituximab could be an effective treatment for pemphigus vulgaris. Consequently, there is a need for a larger, confirmatory, randomized, multicenter trial.
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  • 文章类型: Case Reports
    我们介绍了一名患有大疱性IgA血管炎的儿童。因为皮肤活检显示表皮囊泡伴有中性粒细胞浸润和真皮各层的白细胞碎裂性血管炎,IgA沉积和组织嗜酸性粒细胞增多,中性粒细胞广泛的真皮浸润可能导致了我们患者的水疱爆发和组织嗜酸性粒细胞增多。
    We present a child with bullous IgA vasculitis. Because skin biopsy showed epidermal vesicles with neutrophil infiltration and leukocytoclastic vasculitis in all layers of the dermis, with IgA deposits and tissue eosinophilia, extensive dermal infiltration of neutrophils may have led to both blistering eruptions and tissue eosinophilia in our patient.
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  • 文章类型: Case Reports
    Pemphigus foliaceus is a superficial vesiculobullous disease that typically presents with widespread lesions. Localized presentations are less frequent, and they typically occur in middle-aged patients, following exposure to topical medications, and later on, become more disseminated. We present a case of a 19-year-old female with a localized presentation of pemphigus foliaceus unrelated to previous topical medications, that was a diagnostic and therapeutically challenging case. We also discuss the literature on localized cases, differences in presentations and responses to various treatment modalities.
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