A systematic review on efficacy, safety and treatment durability of intravenous immunoglobulin in autoimmune bullous dermatoses: Special focus on indication and combination therapy.

Autoimmune bullous diseases (AIBDs) are a group of rare blistering dermatoses of the mucous membrane and/or skin. The efficacy, safety and treatment durability of intravenous immunoglobulin (IVIg) as an alternative treatment should be explored to systematically review the available literature regarding treatment outcomes with IVIg in AIBD patients. The predefined search strategy was incorporated into the following database, MEDLINE/PubMed, Embase, Scopus and Web of Science on 18 July 2022. Sixty studies were enrolled using Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines. The use of IVIg alone or combined with rituximab was reported in 500 patients with pemphigus, 82 patients with bullous pemphigoid, 146 patients with mucous membranes pemphigoid and 19 patients with epidermolysis bullosa acquisita. Disease remission with IVIg therapy and RTX + IVIg combination therapy were recorded as 82.8% and 86.7% in pemphigus, 88.0% and 100% in bullous pemphigoid and 91.3% and 75.0% in mucous membrane pemphigoid, respectively. In epidermolysis bullosa acquisita, treatment with IVIg led to 78.6% disease remission; no data were available regarding the treatment with RTX + IVIg in this group of patients. Among all the included patients, 37.5% experienced at least one IVIg-related side effect; the most common ones were headaches, fever/chills and nausea/vomiting. The use of IVIg with or without rituximab had a favourable clinical response in patients with AIBDs. IVIg has no major influence on the normal immune system, which makes its utilization for patients with AIBDs reasonable.

Dermatologists Communication Skills and Performance From Point of View of Patients.

INTRODUCTION: Effective communication is an important issue in patient-doctor interaction which is even more important regarding chronic disorders which need a strong relationship between patient and clinician for better compliance and optimal control disease. OBJECTIVES: This study was aimed to develop the best cultural adapted Persian version of the 28-item Calgary- Cambridge Observation Guide (CCOG) questionnaire. METHODS: In this descriptive-analytic study, data collected from 400 patients attending the outpatient dermatology clinics of 3 major hospitals in Tehran using the modified Persian version of CCOG questionnaire, both before and after being visited by dermatologists. RESULTS: The difference of CCG scores for all of questions, except for questions number 1,16 and 22, was statistically significant. The highest score, both before and after visiting, attained by question regarding being respectful. The lowest scores for necessary behavior and the adequate amount of execution were related to questions number 3 (Introducing self) and 4 (Introducing role), respectively. Age and educational level of patients were significantly correlated with their expectations regarding communication skills of clinician. CONCLUSIONS: This study showed the acceptable validity of modified Persian version of CCOG-24 item questionnaire. Our findings also demonstrated that there was a significant difference between what patients expected from a dermatologist and the manner they actually were treated regarding physician communication skills.

Reliability and validity of the Persian version of psoriatic arthritis screening questionnaire.

Background: The Early Arthritis for Psoriatic Patients (EARP) questionnaire is a fast and simple way to screen psoriatic arthritis. This study was carried out to investigate the diagnostic accuracy of the Persian version of the Early Arthritis for Psoriatic Patients (P-EARP) questionnaire. Methods: A total of 100 psoriasis patients responded to the questionnaire after the translation procedure (translation, back translation). After determining the validity of the questionnaire, the diagnostic accuracy of the P-EARP questionnaire was assessed using the ROC curve (receiver operating characteristic curve). Internal and external reliability of the questionnaire were also evaluated by statistical tests. Results: In assessing the reliability of the questionnaire using test-retest, correlation coefficient (r=0.994, p <0.001) and Cronbach's alpha (α = 0.85) were obtained. The P-EARP questionnaire had a sensitivity and specificity of 90.48% and 96.55% in ROC analysis, respectively, and cutoff point 3 was regarded as the cutoff point of the P-EARP questionnaire like the original version of the questionnaire (EARP). Conclusion: The results of this study showed that the P-EARP questionnaire had high sensitivity and specificity for the identification of psoriatic arthritis. The P-EARP questionnaire is an appropriate screening tool for the identification of psoriatic arthritis in the dermatology clinics.

Kaposi's sarcoma in a patient with pemphigus vulgaris mimicking exacerbation of pemphigus.

BACKGROUND: Kaposi's sarcoma (KS) is a rare multifocal angiogenic tumor often seen in immunocompromised setting such as acquired immunodeficiency syndrome (AIDS) or organ transplantation recipients. Pemphigus vulgaris (PV) is a rare blistering disorder with mucocutaneous involvement for which immunosuppressive therapy has long been the core of treatment. Iatrogenic form of KS has been reported infrequently in pemphigus patients as a result of long-term immunosuppressive therapy. CASE: We describe a 39-year-old male patient with confirmed diagnosis of PV who developed KS after receiving immunosuppressive agents for his pemphigus. KS was initially localized to the oral cavity with features mimicking exacerbation of his pemphigus. CONCLUSION: This interesting case of KS suggests that dermatologists visiting patients with pemphigus with discomfort in the oral cavity should have a high degree of awareness and consider other differential diagnoses along with merely an exacerbation of PV.

Drug Adherence and its Association with Health Literacy Among Patients with Autoimmune Bullous Diseases.

Background: Despite the emergence of novel medications, poor drug adherence is an obstacle toward disease management in patients with autoimmune bullous dermatoses (AIBDs). Objectives: We sought to evaluate medication adherence among patients with AIBDs and determine the impact of health literacy on adherence. Methods: We performed a cross-sectional survey of patients with AIBDs who attended Razi Hospital from May to October 2021. Drug adherence and health literacy were assessed using the Morisky Medication Adherence Scale-8 (MMAS-8; scored 0-8) and Health Literacy for Iranian Adults (HELIA; scored 0-100) questionnaires, respectively. Multivariable ordinal regression, including the covariates age, sex, education level, and annual income, were employed for analyses. Results: Two hundred participants with a mean±standard deviation (SD) age of 50.3±13.5 years were recruited. The female:male ratio was 1.2. About half (53%) of the patients reported good adherence (MMAS-8 score of 8) to their AIBD medications. Moreover, limited health literacy, with a mean±SD score of 57.8±25.8, was noted. Multivariable ordinal regression indicated that literacy score was significantly associated with good drug adherence (odds ratio [OR]: 0.11 per 1 score of health literacy increase, 95% confidence interval [CI]: 0.09-0.14). Conclusion: These findings showed suboptimal drug adherence and health literacy of patients with AIBDs. Improving patient health literacy might be a way to increase drug adherence.

Rituximab Treatment and Improvement of Health-Related Quality of Life in Patients With Pemphigus.

Pemphigus is an autoimmune blistering disease that can negatively affect patients' lives. Assessing the impact of treatment from a patient's perspective using outcome assessment measures is important and relevant in trials of new pemphigus treatments including rituximab (RTX). We sought to evaluate the effect of RTX on health-related quality of life (HRQOL) in pemphigus patients and peruse the clinical relevance of the patient-reported outcomes. A retrospective cross-sectional study was designed with 96 pemphigus patients given RTX either 3 months earlier or in the last 2 weeks. The treatment was evaluated by patients using HRQOL assessment tools: 36-Item Short Form Survey (SF-36) and Dermatology Life Quality Index (DLQI). Another patient-reported assessment was the patient global assessment (PGA). We found that RTX administration in pemphigus patients led to rapid and notable improvement in HRQOL and patient-assessed measures.

Various aspects of the relationship between vitiligo and the COVID-19 pandemic or SARS-CoV-2 vaccines: Clinical pearls for dermatologists.

BACKGROUND: Coronavirus disease 2019 (COVID-19) has given rise to several new onset or exacerbated dermatologic disorders including vitiligo. AIM AND METHOD: Here, we present different aspects of relationship between SARS-CoV-2 infection or its associated vaccines and vitiligo and aim to provide solutions to overcome the potential challenges. RESULTS AND CONCLUSION: In brief, as the benefits overweigh the risks and since vaccine-triggered de novo or flares of vitiligo are uncommon and benign, these patients are recommended to get SARS-CoV-2 vaccines. Moreover, in individuals with previously recognized vitiligo, who are at risk of developing SARS-CoV-2 infection or those who are currently infected, special dermatologic consultation is needed in order to balance the immunosuppressive agents in their therapeutic regimen to prevent COVID-related morbidity and mortality.

Characteristics associated with quality of life in the early stages of Mycosis Fungoides.

Background: Mycosis fungoides (MF) is a lymphoproliferative disorder characterized by skin-homing atypical lymphocytes. This study aimed to evaluate the quality of life (QoL) in MF patients in the early stages of the disease and the associated factors using the Dermatology Life Quality Index (DLQI). Methods: Thirty MF patients (21 females/9 males) with a mean age of 46.73±15.9 years and in the early stages of the disease (26 stage Ι and 4 stage ΙΙ) were enrolled and were asked to fill the DLQI questionnaire. Results: The mean DLQI score was 9.93±5.89. The QoL was largely affected by the disease in near half of the patients (46.7% of patients had a DLQI score11-20). QoL was significantly correlated with educational level and was more impaired in patients with lower educational status (DLQI score spearman rho=-0.382, P=0.037). QoL was not associated with sex, age, disease stage and disease duration. The symptom and feeling dimension of DLQI was significantly more impaired in patients with both patch and plaque compared to patch only (spearman rho= 0.397, P= 0.03). Conclusion: This study demonstrates how largely patients' QoL is influenced in the early stages of MF, especially in patients with lower educational levels.

Two asymptomatic facial erythematous macules in an adult male.

Pemphigus is a group of autoimmune bullous disorders with different types. Pemphigus foliaceous (PF) is a difficult-to-diagnosis disorder which shares clinical features with many dermatoses. We hereby, present an interesting case of PF which serves as a reminder for clinicians that pemphigus is not always a serious condition with rapid extension of lesions and it could have a very limited and benign form.

Ocular manifestations of psoriasis: An inflammatory disease beyond the skin.

Background: Psoriasis is a chronic inflammatory disorder, mainly involves skin. Aims: To evaluate the prevalence of ocular manifestations in Iranian patients with psoriasis, compared to healthy controls. Materials and methods: Forty psoriasis patients and 40 age- and gender-matched healthy controls were enrolled in the study and underwent a comprehensive ophthalmologic assessment. Results: Only meibomian gland dysfunction was significantly more common among patients with psoriasis, compared to control group (p value: 0.011). Regarding intraocular pressure (IOP), the mean values for both patients and healthy controls were within the normal range and mean IOP in patients was even lower than normal controls, although this difference was significant only for left eye (p value: 0.049). A strong positive correlation between PASI and tear meniscus height for both right and left eyes (p value: 0.005, r: 0.44 for OD and p value: 0.003, r: 0.46 for OS.) was noted. Meibomian gland dysfunction was also positively correlated with disease duration for right and left palpebras (p: 0.04, r: 0.31 for both). Conclusion: Psoriasis can lead to meibomian gland dysfunction, especially in patients with long-lasting disease. Hence, dermatologists and general practitioners should be vigilant in this regard when visiting psoriasis patients, especially those who have higher PASI values or long-lasting disease.

COVID-19 vaccination in children as a global dilemma through an ethical lens: A retrospective review.

Background and Aims: COVID-19 pandemic led to a need to rapidly vaccinate as many people as possible. Children are an important part of the population with different characteristics which vaccinating them is a matter of great importance as it should be decided considering all aspects and ethics. Here, we present different aspects of COVID vaccination in children including the potential challenges. Methods: We searched on PubMed, Google Scholar and Scopus in this regard, and all of the relevant papers published until June 28, 2021 were included if we could access their full-texts. Results: We found various expert opinions in this regard and tried to summarized them. Saving lives has similar ethical value as preventing evitable adverse event. Accordingly, mandating the children to receive the SARS-CoV-2 vaccine, needs risk-benefit weighing with special consideration of ethical challenges. Conclusion: Considering the vast range of benefits resulted from pediatric vaccination both for the children and the community, implementing the program in a scientific manner and also with the least financial expenses for the families seems to be reasonable and makes it both ethical and moral.

Unmasking sarcoidosis following SARS-CoV-2 vaccination: A case report.

Sarcoidosis is an inflammatory and granulomatous disorder, developed due to dysregulation between immune response and certain environmental antigens. We hereby report an interesting case of sarcoidosis following COVID-19 vaccination (COVIran Barekat), which presented with inflammation of previous tattoo sites as well as the development of erythema nodosum and systemic lymphadenopathy, suggested a possible link between the COVID vaccination and dysregulation of the inflammatory process and served as a reminder for clinicians to have enough vigilance before proposing a vaccine booster to these patients.

A pure Cutaneous Rosai-Dorfman disease: case report and a review of the literature.

Background: Cutaneous Rosai-Dorfman disease (CRDD) is a rare variant of benign histiocytic proliferative disorder limited to the skin. The underlying etiology is still unclear, but it had been claimed that infections, immunodeficiencies, and autoimmune disorders might have a role in the etiology of this disorder. The characteristic presentation of RDD is lymphadenopathy due to abnormal production and accumulation of histiocytes in lymph nodes; however, the extra-nodal areas could also be affected, such as cutaneous. Herein, we presented a 45-year-old Iranian woman presented with an atypical pure cutaneous Rosai Dorfman disease, in addition to a summarized list of atypical cases of RDD that are reported as pure cutaneous RDD with atypical presentation. Case presentation: Herein, we presented a 45-year-old woman who referred to us with an ulcerative nodule with a size of 5×5 cm on her buttock, gradually growing over one year. After the primary evaluations, a biopsy specimen was obtained, and histologic studies revealed a dense cellular infiltrate involving the dermis and the subcutis, which was composed of abundant sheets of large histiocytes with admixtures of lymphocytes, plasma cells, neutrophils, and eosinophils within their cytoplasm -known as the emperipolesis phenomenon. The immunohistochemistry staining was positive for S100 and CD68 and negative for CD1a. Conclusion: The diagnosis of CRDD was confirmed based on these histopathological findings.

Effects of Herbal Medicines on the Prevention and Treatment of Contact Dermatitis: A Systematic Review.

Background: Contact dermatitis (CD) is a common inflammatory disease of the skin. CD has a complex and multifaceted treatment, and one of the main components of CD treatment is inflammation management. We summarized the clinical trials exploring the effects of herbal medicine on patients with CD. Methods: A systematic review was performed by searching four databases. Clinical trials in English investigating the effect of herbal medicines on CD prevention and treatment published from 2010 to 2020 were reviewed. This study was conducted based on the PRISMA guidelines. Results: Nine clinical trials examining the effects of herbs on CD were identified. A total of 450 patients participated in these studies. Herbal medicines used in the reviewed studies were in various forms. Almost all the studies showed that these herbs were useful in preventing and treating CD. Regarding the side effects of the herbal medicines, few complications such as erythema and papules as well as positive chronotropic effects were reported. Conclusion: Evidence showed that herbal medicines were effective in preventing and treating CD by reducing the inflammation level and increasing antioxidant defense. However, the number of clinical trials was low to draw definitive conclusions. Moreover, the studies were not homogeneous and differed regarding methodology, evaluation tools, and quality. Also, in these studies, the interactions and safety of the herbal medicines were not considered adequately. Therefore, well-designed evidence is required to draw definitive conclusions in this regard.

COVID vaccine recommendations in dermatologic patients on immunosuppressive agents: Lessons learned from pandemic.

Since SARS-CoV2 vaccines were approved without enough long-term monitoring due to emergent situations, some issues have been raised about timing and protocol of receiving them by patients treated by different immunosuppressive agents. Here, we present different aspects of SARS-CoV-2 vaccination in such patients in the field of dermatology. In brief, SARS-CoV-2 vaccination is recommended in all dermatologic patients, regardless of their disorders and therapeutic regimens. Nevertheless, special considerations should be given to the immunosuppressive therapy and its association with vaccination timing due to the decreased immunogenicity of vaccines in this setting. Novel biologic immunotherapies are advantageous over conventional systemic therapies not only in their safety and selective functions but also in this aspect that many of them do not affect vaccines immunogenicity.

A systematic review and clinical atlas on mucocutaneous presentations of monkeypox: with a comprehensive approach to all aspects of the new and previous monkeypox outbreaks.

Monkeypox is a zoonotic disease, which is endemic in the central and west African regions, which has re-emerged, currently causing an outbreak as of May 2022. In this systematic review, we aimed to characterize the current face of the disease, with a detailed categorization of the mucocutaneous, as well as the systemic symptoms of the disease. We searched four main online databases with the keywords "monkeypox" and "Orthopoxvirus". A total of 46 articles were included, with a cumulative number of 1984 reports of confirmed cases (48 from case reports and 1936 from case series and observational studies). Cases are predominantly men who have sex with men (MSM), who are mostly in their 30s, with history of unprotected sexual contact or international travel. Among mucocutaneous manifestations, anogenital lesions were the most commonly observed, followed by lesions on the limbs, face, trunk, and palms or soles. Among lesion types, vesiculopustular lesions, vesicular-umbilicated lesion, pseudo-pustular lesions and ulcers were the most common. Lesions were mainly asynchronous, with less than 10 lesions presenting on each patient. Almost all patients reported systemic manifestations of the illness as well, mainly with fever, lymphadenopathy, fatigue, myalgia, headaches, pharyngitis and proctitis. We've compared these characteristic idiosyncratic findings of the new outbreak with the previous outbreaks. We've also gathered and categorized images from our included studies to make a "clinical atlas" for this "new" face of the disease, which can be of utmost importance for clinicians to be familiarized with, and have monkeypox in their differential diagnoses. This article is protected by copyright. All rights reserved.

COVID-19 vaccines and filler reactions: Should it be considered as a concern?

BACKGROUND: Any implant or external material used in the body tissues can potentially be followed by autoimmune or inflammatory reactions. With the global vaccination program against COVID-19, the reports of tissue filler reactions would be increasingly demonstrated. AIM: To summarize the data regarding COVID vaccination and filler reactions. METHOD: We reviewed the existing data in this regard through searching on PubMed, Google Scholar and Scopus. All of the relevant papers published until March 2022, which we could access to their fulltexts were included. RESULTS: Here, we summarized the data regarding COVID-19 vaccination and filler reactions and discussed its etiopathogenesis, management, and importance. CONCLUSION: Although the end of pandemic was announced, the necessity of continuing COVI-D19 vaccination in future mandates gathering data regarding safety of vaccines.

Coexistence of Pemphigus Vulgaris and Lichen Planus following COVID-19 Vaccination.

SARS-CoV-2 vaccines were approved without long-term monitoring due to emergent situation and might have several side effects. Herein, we describe the first case with development of both LP and PV following COVID-19 vaccination. Immunological alteration due to COVID-19 vaccination and its potential role in triggering autoimmune disorders were also dealt with.