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.

Efficacy and safety of oral pioglitazone in the management of lichen planopilaris in comparison with clobetasol: A randomized clinical trial.

Lichen planopilaris (LPP) is a scarring alopecia for which no treatment with remarkable effect has been identified. Pioglitazone has been reported as a possible therapeutic option. To compare the efficacy and safety of pioglitazone with clobetasol in LPP. This randomized, double-blind, parallel-group was conducted at Razi hospital. Patients were treated either with pioglitazone 15 mg/daily or clobetasol lotion 0.05% once at night for 6 months. Patients were visited every 2 months to assess the lichen planopilaris activity index (LPPAI) and record probable adverse events. Forty patients (mean age: 43.6 years; 62.5% female) were randomized 1:1. The mean of LPPAI at baseline and last session were 4.68 ± 1.97 and 2.59 ± 0.97 in the clobetasol group and 5.01 ± 1.71 and 3.04 ± 1.36 in the pioglitazone group, respectively. Both treatments significantly decreased the LPPAI over the two-month interval visits (p < 0.001). No significant difference in the LPPAI reduction was detected between groups. Regarding the safety profile, three clobetasol-treated patients developed folliculitis, and two in the pioglitazone group developed mild headaches. Pioglitazone effectively controlled the signs and symptoms of the LPP with no serious side effects. It can be considered a treatment option for LPP, although it was not superior to clobetasol.

Adverse outcome and severity of COVID-19 in patients with autoimmune bullous diseases: A historical cohort study.

The ongoing COVID-19 pandemic has raised concerns regarding the outcome of this infection in patients with autoimmune bullous dermatoses (AIBDs) due to effect of drugs used to treat these disorders. This investigation was performed from the onset of the pandemic to June 1, 2021. Patients with AIBDs who contracted COVID-19 were evaluated. A generalized linear model was employed to find the predictors of severe COVID-19 among patients with AIBDs. Ninety-three patients with AIBDs with a mean age of 50.3 years were evaluated. The most COVID-19 related symptoms were tiredness (76.3%) myalgia (69%), and cough (63.4%). During follow-up, the rate of hospitalization and death were 45.2% and 4.3%, respectively. Previous comorbidities (ß = 0.61) and mean prednisolone dosage above 10 mg/day in the last 3 months (ß = 1.10) significantly increased COVID-19 severity. Also, vaccination against SARS-CoV-2 (ß = -1.50) and each passing month from the last rituximab dose decreased severity (ß = -0.02). Notably, 19.3% of the patients developed AIBD flare-ups following COVID-19 infection. Higher prednisone dose and the shorter interval from the last rituximab infusion were determinants of severe COVID-19. Physicians should assess the risk versus the benefits when prescribing the medications. Moreover, vaccination could successfully attenuate COVID-19 severity.

Applicability of glucocorticoid toxicity index in pemphigus: Comparison between two groups of rituximab-treated and rituximab-naïve patients.

Rituximab (RTX) combined with short-term glucocorticoids (GC) is an effective therapeutic option for pemphigus. The newly developed Glucocorticoid Toxicity Index (GTI) tool provides the possibility to measure GC toxicities over time. To compare 1-year GTI between two groups of RTX-treated and RTX-naïve patients with pemphigus. The responsiveness of the GTI was also investigated. A prospective cohort of 129 adults with newly diagnosed pemphigus was conducted. GC-related toxicities were assessed at 3-month intervals according to Composite and Specific lists of the GTI. Of the patients, 76.7% (n = 99) received RTX. Throughout the time intervals, RTX-treated patients had lower GTI compared to RTX-naïve ones (p = 0.036). The mean GTI at 1-year was 34.3 in the RTX-treated group and 50.8 in the RTX-naïve group (p = 0.04). The most commonly observed GC-related toxicity was neuropsychiatric manifestations for 34% (224 events). The relapse rate of RTX-treated patients (1%) was significantly lower than RTX-naïve patients (10%) (p = 0.037). The GTI showed no correlation with cumulative GC consumption in both groups (p > 0.05, both). Patients treated with GC alone had remarkably higher GTI than patients treated with GC plus RTX. The GTI is an applicable tool to quantitatively capture GC toxicities at the patient level in pemphigus.

Does topical minoxidil at concentrations higher than 5% provide additional clinical benefit?

Minoxidil solution is used for the treatment of androgenetic alopecia (AGA). Minoxidil 5% solution has been shown to have superior efficacy to minoxidil 2% solution, it is unknown whether concentrations of minoxidil > 5% provide additional clinical benefit. We performed a review of the literature to examine the evidence on the efficacy and safety of higher concentrations of minoxidil in AGA. A search of the PubMed database was performed using the keywords 'minoxidil', 'androgenic alopecia' and 'pattern hair loss'. Relevant articles, including clinical trials, other clinical studies, case series and case reports published in English were considered for review. In addition, relevant references from the bibliography section of the retrieved articles were also reviewed.

Comparison of the efficacy and safety of 308-nm excimer laser with intralesional corticosteroids for the treatment of alopecia areata: A randomized controlled study.

BACKGROUND: Corticosteroids have been the mainstay of treatment for alopecia areata (AA). Recently, the 308-nm excimer laser has been proposed for treating AA. OBJECTIVES: To compare the efficacy and safety of excimer laser with intralesional corticosteroid (ILCS) in AA. METHODS: Patients with at least two alopecic patches were randomly assigned to receive weekly excimer laser treatments or monthly injections of ILCS. Photographs and trichoscopy images were examined at baseline, the last treatment session, and after one month of follow-up. The hair regrowth score was evaluated on a 6-point scale. RESULTS: Sixteen patients with 99 alopecic patches completed the study. At the last treatment session, the mean score of hair regrowth for the laser was significantly lower than the ILCS (p = 0.003). However, after a month of follow-up, the difference was not statistically significant (p = 0.148). Positive response in hair regrowth (≥50%) was achieved in 47% of laser-treated patches and 66% in ILCS-treated ones. Four (25%) and 8 (50%) patients experienced severe adverse events of laser and ILCS, respectively. CONCLUSIONS: The excimer laser was safe and effective in AA. The effect of laser on hair regrowth might be delayed as compared with ILCS.

Pigmented purpuric dermatoses versus purpuric mycosis fungoides: Clinicopathologic similarities and new insights into dermoscopic features.

BACKGROUND: The diagnosis of purpuric mycosis fungoides (PMF) is often challenging to be clinically differentiated from inflammatory diseases such as pigmented purpuric dermatosis (PPD). Dermoscopy as a non-invasive method can be employed for the visualisation of features invisible to the naked eye. OBJECTIVES: This study aimed to survey the dermoscopic findings of PMF in comparison with PPD. METHODS: Forty-one patients with an established diagnosis of PMF (n = 28) and PPD (n = 13) were prospectively recruited. Dermoscopic images were taken by FotoFinder Medicam 1000 (FotoFinder Systems GmbH, Bad Birnbach, Germany). RESULTS: Characteristic dermoscopic patterns consisting of fine short linear vessels (35.7%, P = 0.017) and spermatozoa-like structures (50%, P = 0.014) were found to be significantly more common in PMF lesions, while PPD lesions were typified by erythematous globules (76.9%, P = 0.01), in the background colour of dull red (61.5%, P = 0.01) and reticular pigmentation (61.5%, P = 0.044). CONCLUSIONS: This study showed the usefulness of dermoscopy for the diagnosis of PMF and PPD cases. Studies with long-term follow-up are needed to affirm the value of these dermoscopic patterns in the differentiation between the two entities.

Clinical and histopathological evaluation of cutaneous angiofibromas.

BACKGROUND: Angiofibromas are common benign skin lesions that are diagnosed clinically and histopathologically. Fibrous papule (FP) is one of the clinical types of angiofibromas located primarily on the nose. METHODS: The cases with the confirmed diagnosis of angiofibroma were enrolled. Demographic features of the patients and clinical characteristics of the lesions were extracted. Moreover, histopathological features of the available slides were microscopically reviewed. RESULTS: From 83 cases of cutaneous angiofibromas, 57 (69%) had lesions consistent with FP. The mean age at diagnosis was 37.7 ± 16.3 years and the most common site of involvement was the nose (53% of cases). Regarding the histopathological evaluation of 71 slides, most cases represented vascular changes (97%) and dermal fibroplasia (87%). Melanin incontinence (41%), acanthosis (31%), and lichenoid features (25%) were other common histopathological findings. Moreover, a sparse inflammatory cell infiltrate was present in 68% of cases. The most prevalent inflammatory cells were lymphocytes (61%) and histiocytes (34%). CONCLUSION: This study presented the histopathological findings of a series of cutaneous angiofibromas. Dermatopathologists should be aware of these features, especially those that are less common, to better diagnose and differentiate them from malignant neoplasms.

Efficacy and safety of hydrogen peroxide topical solution in treating actinic keratosis: A randomized controlled trial.

Actinic keratosis (AK) is a precancerous skin lesion associated with the development of squamous cell carcinoma. Approved topical treatments for AK are unmet needs. To evaluate the safety and efficacy of hydrogen peroxide topical solution 35% (H2 O2 ) with cryosurgery for the treatment of AKs. Eligible subjects were randomly assigned to receive H2 O2 and cryosurgery on each side of their scalp/face. Patients were assessed at baseline and after 3 months of follow-up. Efficacy was evaluated based on the Actinic Keratosis Area and Severity Index (AKASI) and dermoscopic assessments. Fifteen patients with 98 lesions completed the study. At the follow-up visit, the mean improvement of AKASI from baseline was 1.7 ± 0.9 for H2 O2 and 1.7 ± 0.8 for cryosurgery (p < 0.001, both). Both treatments significantly improved dermoscopic features of yellow scale, white scale, linear-wavy vessels, pigmented dots, and rosette at the end of the 3-month follow-up. Complete dermoscopic response occurred in 57.7% and 73.9% of lesions treated with H2 O2 and cryosurgery, respectively (p > 0.05). Regarding safety, hypopigmentation occurred more frequently in the cryosurgery group (28.3% vs. 9.6%; p = 0.017). Moreover, patients endured more pain with cryosurgery application than H2 O2 (p < 0.001). The effect of H2 O2 solution was comparable to cryosurgery. This treatment was well tolerated and had a favorable safety in patients with AKs.

The Impact of Facial Asymmetry on the Surgical Outcome of Crooked Nose: A Case Control Study.

BACKGROUND: Facial asymmetry is considered a reason for patient dissatisfaction with the outcome of rhinoplasty. OBJECTIVES: The aim of this study was to evaluate the impact of facial asymmetry on patient postoperative satisfaction with crooked nose and to investigate the relationship between visual perception of asymmetry and anthropometric measurements. METHODS: In this retrospective study, 61 rhinoplasty patients with crooked noses were assessed. Utilizing frontal view photos, pre- and postoperative nasal deviation angles were calculated. The patients were divided into 2 groups based on the visual presence of facial asymmetry. Moreover, anthropometric characteristics of the face were evaluated utilizing facial soft tissue landmarks. Patient satisfaction with surgery outcomes, including both aesthetic and functional aspects, was assessed employing the Persian version of the Standardized Cosmesis and Health Nasal Outcomes Survey. RESULTS: Based on the observations, 19 (30.2%) and 44 (69.8%) patients had I-type and C-type noses, respectively. In both groups, the deviation angle decreased significantly postoperatively (P < 0.001). Regarding the subjective evaluation of facial asymmetry, 22 (34.9%) and 41 (65.1%) cases had symmetric and asymmetric faces, respectively. Anthropometric measurements were also consistent with visual assessments of asymmetry. Satisfaction scores were significantly higher after surgery in all patients; however, there was no significant difference in the mean aesthetic improvement between symmetric (15.83 ± 2.68) and asymmetric faces (15.23 ± 4.46) (P = 0.531). The power of study was 97.8%. CONCLUSIONS: Rhinoplasty in patients with deviated noses and asymmetric faces may have comparable results with symmetric ones.

Rituximab in the Treatment of Epidermolysis Bullosa Acquisita: A Systematic Review.

Objective: Epidermolysis bullosa acquisita (EBA) is a rare dermatosis of the mucous membrane and/or skin. Employing biologic treatment modalities, specifically rituximab (RTX), have become pivotal measure in treating patients with blistering diseases. This study aims to summarize the current evidence on the safety and efficacy of RTX in EBA. Methods: An extensive search was performed in MEDLINE/PubMed, Embase, Scopus, and Web of Science databases until the end of August 19th, 2023. Two independent reviewers screened the papers, and collected data. Two hundred thirty-three studies were screened using Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines. Results: Thirty-one studies were enrolled. The most common reason of RTX administration in patients with EBA was recalcitrant diseases. Clinical response and disease remission was recorded as 92.7 percent (63 patients) and 73.8 percent (45 patients) of the patients, respectively. A relapse rate of 39.5 percent (15 patients) in the mean follow-up of 23.0 months was reported in the studies. Of the patients, 28.2 percent (11 patients) experienced RTX-related side events, mostly mild and transient infusion reactions. Conclusion: The results of this systematic review demonstrated that RTX is safe and effective in patients with EBA. This biological treatment modality can be routinely used in managing EBA.

Lichenoid mycosis fungoides: Report of a case with lichen planus-like histopathologic features.

Key Clinical Message: Mycosis fungoides is a diagnostic challenge. Herein, we report a case with marked lichenoid features in pathology assessments. After several biopsies and clinicopathologic correlation, the diagnosis of lichenoid mycosis fungoides was made. Abstract: Mycosis fungoides (MF) is a great imitator and mimicks other dermatoses clinically and histopathologically. We report a 61-year-old patient with 5-year history of generalized violaceous patches and plaques. His biopsy revealed a marked lichenoid band-like infiltrate of inflammatory cells along the basal layer with basal layer vacuolar changes; the diagnosis of lichen planus was first made histopathologically. Several biopsy specimens, clinicopathologic correlation, and immunohistochemistry findings confirmed the diagnosis of lichenoid MF. Awareness of peculiar histopathologic findings of MF is essential to avoid a potential misdiagnosis. When in doubt, multiple biopsies with other diagnostic methods should be employed.

Perception of timbre in adult Cochlear implant users: Comparison of Iranian and Western musical instruments.

OBJECTIVES: Cochlear implants (CI) have dramatically improved speech perception for patients with sensorineural hearing impairment. However, listening to music is a great challenge for them. This study examined the perception and appraisal of Iranian musical instruments comparing with similar Western instruments. METHODS: Eleven adult CI users and 25 normal hearing (NH) individuals participated in this study. Musical stimuli of three commonly heard instrument pairs were prepared. Participants were asked to identify the instruments and rate their appraisal on a ten-point Likert scale (0 = dislike very much, 10 = like very much). RESULTS: The instrument recognition rate was 40.6% among the CI users, and the mean appraisal score was 5.2 ± 2.7. NH listeners had none significant higher scores on both tasks with a recognition rate of 50.0% and the mean appraisal score of 6.9 ± 1.5. Iranian instruments were more recognized in both groups. Regarding their appraisal, the mean score for both types was almost equal in the NH group, while CI users more appraised Iranian instruments. CONCLUSION: In addition to better recognition of Iranian instruments, they were particularly better appraised in the CI group. Iranian instruments provide suitable musical pieces for CI recipients that can be considered in rehabilitation programs.

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.

Burden of pemphigus vulgaris with a particular focus on women: A review.

Pemphigus vulgaris is a chronic autoimmune mucocutaneous blistering disorder. Apart from the disease itself, other aspects of patients' life, including psychological, social, and financial, can be affected. Women are particularly more disposed to the impact of the disease due to their physiological characteristics, the specific periods of pregnancy and lactation as well as their social and familial role. In this review, we summarized the burden of pemphigus vulgaris on various aspects of women's lives. It is essential to understand these problems and provide appropriate support for patients with such a burdensome disease.

Music Appreciation of Cochlear Implant Users versus Normal Hearing Individuals.

Introduction: Cochlear implants (CI) provide speech perception for patients with sensorineural hearing impairment; nonetheless, listening to music is a daunting challenge for them. The present study aimed to compare Iranian CI users and normal hearing (NH) controls in terms of musical habits and appreciation and investigate the possible effect of background variables. Materials and Methods: A total of 37 CI users who underwent surgery at least 18 months before the study and 59 NH listeners were enrolled in this study. The participants were assigned to two age groups: group A (patients ≥15 years old) and group B (patients <15 years old). They were asked to complete the questionnaires to assess their music engagement. Results: In group A, the mean score of music importance was significantly higher in CI users (8.7±2.1), as compared to that in NH subjects (5.8±2.3) (P=0.005). Participation in professional musical training and singing with music was not significantly different between the groups. In group B, the mean score of desire for music was not significantly different between CI users (8.2±1.8) and NH subjects (7.7±2.0). They participated in professional musical training and had a reaction to music almost equally. Singing with music was significantly less common in the CI group (CI 16[61.5%], NH (40[85.1%]) (P=0.023). Selected background variables had no significant effect on the music tendency and habits of CI users. Conclusions: Iranian CI users tended to have a high level of music appreciation in both adult and children groups. Moreover, CI users and NH controls did not significantly differ in the importance of music, devoted time, participation in musical activities, and musical habits.

Pregnancy outcomes in women with pemphigus exposed to rituximab before or during pregnancy.

Rituximab (RTX) is an effective treatment for pemphigus; however, the drug labeling recommends not to use RTX within 1 year before conception. Objectives: To report pregnancy outcomes of patients with pemphigus who were treated with RTX before or during pregnancy. Methods: We identified 19 pregnancies with RTX exposure before or during pregnancy. All had previously been advised not to get pregnant within 1 year of RTX administration. The cases were categorized into 3 groups of exposure of within 6 months (group A), between 6 and 12 months (group B), and longer than 12 months of conception (group C). The pregnancy outcomes of different RTX exposure intervals were compared. Results: Group A included 9 pregnancies, of which 3 had received RTX accidentally after conception. Group B and C included 4 and 6 pregnancies, respectively. There was no significant difference between the groups regarding pregnancy outcomes. Overall, there were 17 live births, 1 spontaneous abortion, and 1 termination. Of the live births, 3 preterm deliveries and 4 low-birth-weight neonates were noted. Moreover, 1 neonate was hospitalized due to early-onset neonatal sepsis, and 1 had hydronephrosis. Disease flare-up occurred in 5 patients during pregnancy (4 minor and 1 major relapses) and in 5 patients after delivery (3 minor and 2 major relapses). Conclusions: Except for 1 case of neonatal sepsis which survived following medical treatment, no serious relevant adverse pregnancy outcome that could be attributed to RTX exposure before and during early pregnancy in women with pemphigus was detected. Nevertheless, RTX should not be administered within 1 year before planned pregnancy, as not enough data is available yet.