Intravenous Immunoglobulin Therapy in Autoimmune Mucocutaneous Blistering Diseases

Intravenous immunoglobulin (IVIg) is a biologic agent that is being increasingly used in the treatment of autoimmune and chronic inflammatory disorders. It is approved by the US FDA for the treatment of primary immunodeficiencies, immune thrombocytopenic purpura, Kawasaki disease, bone marrow transplantation in patients aged over 20 years, chronic B-cell lymphocytic leukemia, and pediatric AIDS. IVIg has been used off-label for several diseases, clinical symptoms and syndromes. Our aim was to determine if there is evidence to support the efficacy of IVIg therapy in autoimmune mucocutaneous blistering diseases (AMBDs). We searched the PubMed database for studies on pemphigus and pemphigoid using the following criteria: (i) English language; (ii) minimum of five patients; (iii) diagnosis based on histology and immunopathology; and (iv) statistical analysis of data for comparison of efficacy provided. We evaluated the data and present information on the number of participants in each study, pre-IV g therapy, indications for the use of IVIg, IVIg protocol (dose and interval) used, concomitant therapies, clinical outcome, follow-up period, and serologic studies. The quality of the evidence presented in this review is at Level A according to the UK National Health Service criteria. Twenty-three studies that were published between May 1999 and April 2010 were identified. One randomized controlled trial was found and all other studies were case series. Data on 260 patients treated with IVIg were analyzed: 191 patients with pemphigus and 69 patients with pemphigoid. Overall, 245 patients showed improvement with IVIg therapy. IVIg demonstrated a corticosteroid-sparing effect. In the studies presented, the incidence of serious adverse effects was not significant. The best available evidence in the literature indicates that IVIg is efficacious and has a good safety profile in the treatment of AMBDs.     

Psychological Therapies in Management of Psoriatic Skin Disease: A Systematic Review

Background

Psoriasis is a chronic, immune-mediated skin disease shown to have a multifaceted relationship with psychological factors. Because these factors have been shown to both worsen and result from psoriasis, an increasing number of studies have sought to investigate the efficacy of various psychological interventions in psoriasis management.

Methods

A systematic review of PubMed^® and Scopus^® databases was performed for studies investigating psychological interventions in psoriasis management published from 1 January 1990 through 4 November 2018. Primary articles published in English and conveying physical treatment outcomes were included, whereas articles not describing clinical outcomes were excluded. Studies supporting intervention efficacy were graded with a level of evidence according to the Scottish Intercollegiate Guidelines Network levels of evidence.

Results

A total of 28 reports studying 27 unique sets of patients receiving psychological therapies in psoriasis management were identified, including three case reports and series and 24 clinical trials, investigating 1522 patients in total. Cognitive behavioral therapy and its variants, biofeedback, meditation and mindfulness-based therapies, hypnosis, music resonance therapy, motivational interviewing, emotional disclosure, and educational and multidisciplinary approaches have been studied in the treatment of psoriasis. Although 16 randomized controlled trials were included in this review, literature is limited by heterogeneity of methodology, analyses, and outcomes. Only 4 of 27 studies (three of which investigated cognitive behavioral therapy) were rated a level of evidence of 1+ or greater. Studies, overall, have sample sizes often < 50 patients, lack follow-up past 12 months, and have attrition rates > 20%.

Conclusions

Based on assigned levels of evidence, the most promising methods of psychological intervention in psoriasis include cognitive behavioral therapy, mindfulness-based therapies, motivational interviewing, and educational and interdisciplinary interventions. Further study is needed to determine the efficacy, practicality, and economic feasibility of these treatment options for patients with psoriasis.

     

Diagnostic Approach to Autoimmune Blistering Diseases Using Immunoblotting with Chemiluminescence

Autoantibodies in sera from patients with autoimmune blistering diseases were detected by immunoblotting with chemiluminescence. This method provided the high sensitivity in the detection of autoantibodies against human epidermal extract. It was useful for detecting low titers of autoantibodies, and identifying small amounts of autoantigens in antigen extracts.     

Neonatal Lupus Erythematosus: A Review

Neonatal lupus erythematosus is an uncommon type of lupus that in addition to cutaneous lesions may have systemic involvement. It is generally a benign condition unless complicated by complete heart block, which is irreversible and which carries a high mortality rate in the first year of life. Patients' mothers often show clinical and/or laboratory features of connective tissue diseases.     

Risk of Parkinson's Disease Among Patients with Psoriasis: A Systematic Review and Meta-analysis

Background:Patients with psoriasis might be at a higher risk of developing Parkinson''s disease (PD) as a result of the detrimental effect of chronic inflammation on the neuronal tissue. This meta-analysis aimed to investigate this risk by comprehensively reviewing all available data.Methods:We conducted a systematic review and meta-analysis of cohort and case–control studies that reported relative risk, hazard ratio, odds ratio, or standardized incidence ratio comparing the risk of PD in patients with psoriasis versus subjects without psoriasis. Pooled risk ratio and 95% confidence interval (CI) were calculated using random-effect, generic inverse variance methods of DerSimonian and Laird.Results:Three retrospective studies and one case–control study met our eligibility criteria and were included in this meta-analysis. The pooled risk ratio of PD in patients with psoriasis versus participants without psoriasis was 1.38 (95% CI, 1.15–1.66). The statistical heterogeneity was low with an I² of 35%.Conclusions:Our meta-analysis demonstrated a statistically significant increased risk of PD among patients with psoriasis.     

静脉注射免疫球蛋白治疗自身免疫性大疱病的现状

静脉注射免疫球蛋白作为一种免疫调节剂可治疗多种免疫相关性疾病。自身免疫性疱病是在皮肤科中使用静脉注射免疫球蛋白最有效的疾病之一。该方法不良反应较少且大多有自限性,为一些常规治疗禁忌或无效的患者提供了安全有效的治疗选择。但其确切的作用机制尚未完全明了,近年来的研究显示可能与抗独特型抗体、中和Fc受体、调节细胞因子网络、影响发病中的细胞凋亡过程等有关。     

自身免疫性表皮下大疱病基底膜带自身抗体的检测

目的 比较免疫印迹（ＩＢ）和盐裂皮肤间接免疫荧光（ＩＩＦ）检测自身免疫性表皮下大疱病（ＳＡＢＤ）基底膜带自身抗体（ＢＭＺ－Ａｂ）的敏感性和特异怀。方法 分另应用ＩＩＦ和ＩＢ技术对９７例ＳＡＢＤ患者血清中ＩｇＧ型或ＩｇＡ型ＢＭＺ－Ｚｂ进行检测。结果 ＩＩＦ法阳性率７５．３％,免疫印迹法阳性率７９．４％,两者在检测灵敏度上无显著性差异。结论 二者均可用于ＳＡＢＤ中ＢＭＺ－Ａｂ的检测,二者联用对于提高Ｂ     

Mosaic Neurofibromatosis Type 1: A Systematic Review

Confusion is widespread regarding segmental or mosaic neurofibromatosis type 1 (MNF1). Physicians should use the same terms and be aware of its comorbidities and risks. The objective of the current study was to identify and synthesize data for cases of MNF1 published from 1977 to 2012 to better understand its significance and associations. After a literature search in PubMed, we reviewed all available relevant articles and abstracted and synthetized the relevant clinical data about manifestations, associated findings, family history and genetic testing. We identified 111 articles reporting 320 individuals. Most had pigmentary changes or neurofibromas only. Individuals with pigmentary changes alone were identified at a younger age. Seventy‐six percent had localized MNF1 restricted to one segment; the remainder had generalized MNF1. Of 157 case reports, 29% had complications associated with NF1. In one large case series, 6.5% had offspring with complete NF1. The terms “segmental” and “type V” neurofibromatosis should be abandoned, and the correct term, mosaic NF1 (MNF1), should be used. All individuals with suspected MNF1 should have a complete physical examination, genetic testing of blood and skin, counseling, and health surveillance.     

Topical Retinoids in Acne Vulgaris: A Systematic Review

Background

Topical retinoids are a first-line treatment for acne vulgaris.

Objective

This systematic review aims to evaluate the efficacy, safety, and tolerability of topical retinoids approved in the United States for the treatment of acne vulgaris.

Methods

A PubMed and Embase search was conducted using the search terms ‘adapalene,’ ‘tretinoin,’ ‘tazarotene,’ and ‘acne vulgaris.’ Selection of articles fit the following inclusion criteria: clinical trials evaluating both efficacy and safety/tolerability of topical retinoids approved in the United States for the treatment of acne vulgaris and published between January 1, 2008 and September 1, 2018. Exclusion criteria included clinical trials involving 20 subjects or fewer, subjects under 12 years of age, and topical retinoid combination therapies with moisturizers or aloe vera. Of 424 search results found, a total of 54 clinical trials were chosen based on selection criteria.

Results

Topical retinoids are superior to vehicle in improving Investigator Global Assessment and Investigator’s Static Global Assessment (24.1–28.8% and 13.3–17.3%, respectively; p < 0.001). A topical retinoid combined with benzoyl peroxide led to IGA improvement compared with vehicle (26.1–34.9% vs 7–11.8%; p < 0.001) at Week 12. Topical retinoid plus an oral antibiotic was superior to vehicle in reducing lesion counts (64–78.9% vs 41–56.8%, p < 0.001). There was no significant difference in efficacy between tretinoin and tazarotene. Tretinoin 0.05% resulted in 62% of patients experiencing AEs compared with adapalene 0.1% (19%) and adapalene 0.3% (40%). More patients receiving adapalene were tolerant of the AEs compared with tazarotene (55.4% vs 24.4%; p < 0.0012).

Conclusions

Topical retinoids are safe and efficacious for the treatment of acne vulgaris. They should be used in combination with benzoyl peroxide to optimize results in patients. The differences in efficacy of topical retinoids appears minor; therefore, the type of topical retinoid is not as important as choosing a particular strength of topical retinoid and combining it with an antimicrobial agent. Adapalene has a superior tolerability profile amongst topical retinoids.