Knowledge, Attitude, and Practice toward Skin Cancer among Patients of Dermatology Clinics and Medical Students/General Practitioners.

This cross-sectional study assessed the knowledge, attitude, and practices (KAP) regarding skin cancer among dermatology clinic patients, medical students, and general practitioners (GPs) in Tehran, Iran. The researchers collected data using a validated questionnaire administered online, measuring KAP on scales of 0-31, 0-16, and 0-28, respectively, with scores above 16, 8, and 14 indicating "good" levels. Of 2243 participants (mean age 28 years), 59.4% had good knowledge, 19.8% had good attitudes, 31.8% had good practices, and 29.8% had good overall KAP. Medical students/GPs scored higher on knowledge and attitudes, while patients scored better on practices. Knowledge, attitudes, and practices were positively correlated in professionals but inversely correlated in patients. The findings suggest that while knowledge was moderate, attitudes and behaviors remained poor, particularly among patients. Immediate interventions are needed to improve attitudes and prevention practices, as public health initiatives must focus on positively influencing both to translate knowledge into meaningful action and find the reasons why good knowledge may not always lead to good practice. These findings underline the need for targeted interventions to bridge the gap between knowledge and preventive behaviors, to effectively reduce the burden of skin cancer in the population.

Mild cognitive impairment in pemphigus.

BACKGROUND: Pemphigus is a group of autoimmune blistering disorders that have been associated with dementia in previous studies. Mild cognitive impairment (MCI) can be the first stage of progression into dementia. The objective of the present study was to evaluate the frequency of MCI in pemphigus patients compared to a control group. METHODS: This case-control study included 80 patients with pemphigus referred to the dermatology clinics of Shohadaye Tajrish and Loghman Hakim hospitals, Tehran, Iran, in 2021. A group of 80 individuals without pemphigus who visited the same clinics for cosmetic consultation or interventions were regarded as controls. Age, sex, marital status, and education were recorded for all participants. Disease duration, medications, and severity were noted for pemphigus patients. The Persian version of the Montreal Cognitive Assessment (MoCA) test was used to assess cognitive function. RESULTS: MCI was significantly more frequent in pemphigus patients than in controls (55% vs. 37.5%, P = 0.026). Furthermore, the total MoCA score was significantly lower in pemphigus patients compared to controls (23.98 ± 3.77 vs. 25.21 ± 3.45, P = 0.032); however, among MoCA's different domains, only the executive functions score was significantly lower in pemphigus patients (P = 0.010). After adjustment, multivariable logistic regression analysis revealed that every 1-year higher education in patients decreased the odds of MCI by 52% (adjusted odds ratio = 0.483, 95% confidence interval 0.326; 0.715, P < 0.001). CONCLUSIONS: The frequency of MCI was found to be significantly higher, and overall scores of the MoCA test, as well as its executive function domain, were significantly lower among pemphigus patients in this study compared to the control group. Additionally, a higher level of education was associated with decreased odds of MCI in pemphigus patients. Identifying pemphigus patients with MCI through the use of the MoCA test can facilitate early intervention, enabling them to seek help and support.

The Combined Application of Bleomycin and Triamcinolone for Treating Refractory Keloids.

BACKGROUND: Triamcinolone acetate injections are considered the first treatment option for keloids, but quite high proportions of keloids either do not respond to triamcinolone or develop recurrence. Beneficial effects of intralesional bleomycin have been recently shown in the treatment of keloids and hypertrophic scars. However, the efficacy of combination therapy using intralesional triamcinolone and bleomycin remains undetermined. OBJECTIVE: The purpose of this study was to evaluate the efficacy of using bleomycin and triamcinolone mixture to treat refractory keloids. MATERIALS AND METHODS: In total, 33 patients with resistant keloids (including 8 men and 25 women) and a mean age of 36.52 years (age range of 18-65 years) were enrolled in this study. A mixture of bleomycin (1 u/cc) with triamcinolone acetonide (13.3 mg/cc) was injected intralesionally into the keloids every 4 to 6 weeks for a maximum of 6 cycles. The clinical improvement was evaluated using the Japan Scar Scale (JSS) and the physician's global assessment of the flattening of the lesions. Side effects were also noted and recorded. RESULTS: In all patients, the total JSS scores decreased significantly after treatment (2.33 ± 1.05), compared with baseline (11.61 ± 2.59), ( p < .001); 26 keloids (78.8%) showed an excellent response (75%-100% flattening), 7 keloids (21.2%) showed a fair response (25%-75% flattening), and 0 keloids (0%) showed a poor response (<25% flattening). Observed side effects were ulceration (33.3%), hyperpigmentation (33.3%), hypopigmentation (15.15%), secondary infection (33.3%), and telangiectasis (15.15%). CONCLUSION: The combined use of bleomycin and triamcinolone offers a promising treatment option for individuals who have not responded well to traditional therapies.

Brain MRI findings in patients with bullous pemphigoid: A case-control study.

Evidence suggests that bullous pemphigoid (BP) is associated with multiple neurological disorders. We aimed to compare brain magnetic resonance (MRI) findings between BP patients and a control group. This case-control study included patients with BP referred to two dermatology clinics during a two-year period. A group of individuals attending the same clinics for cosmetic procedures were selected as controls. First, participants' general information including age, gender, education, weight and underlying disease was recorded. For BP patients, the drugs and the BP Disease Area Index (BPDAI) were recorded as well. Then, all participants underwent brain MRI without contrast. The Fazekas scale, the general cerebral atrophy (GCA) score, and the Medial Temporal lobe Atrophy (MTA) score were used to assess MRI images. Overall, 24 BP patients and 24 controls were evaluated in this study. Both groups were comparable regarding age, gender and education. However, diabetes and hypertension were more frequent in the control group. The mean BPDAI total score was 51.39 ± 68.92 in BP patients and most of them used rituximab (41.7%). None of the participants had MS or Alzheimer MRI patterns. There was no difference between groups in terms of GCA and MTA scores. Furthermore, the frequency of partially empty sella did not differ significantly between BP patients and controls (p = 0.461); nevertheless, grade-3 Fazekas was significantly higher in BP patients compared to controls (25% vs. 0%, p = 0.019). Of note, one BP patient had an epidermoid cyst and another had moderate enlargement of three ventricles. Also, new infarcts were observed in two and old infarcts in four BP patients. Although the majority of abnormal brain MRI findings were more frequent in BP patients compared to controls, only grade-3 Fazekas was significantly higher and acute infarcts were exclusively observed in BP patients.

Efficacy of oral tofacitinib in refractory chronic spontaneous urticaria and urticarial vasculitis.

The treatment of chronic urticaria (CU) is difficult, currently, antihistamines (AH) are the mainstay of treatment, however, up to 40% of patients do not respond to even high (four-fold) daily doses of AH. Tofacitinib is, a small-molecule that blocks JAK1/3 and inhibits intracellular signaling of multiple key cytokines involved in the inflammatory cascade and its beneficial effects were reported in patients with mast cell activation disease but there is no report in patients with urticaria. Here, we present four cases of refractory CU and one case of urticarial vasculitis (UV) that were managed with tofacitinib. Despite the long-term unresponsiveness of various treatments in our patients, the addition of tofacitinib significantly improved the urticarial activity and ultimately led to tapering and discontinuation of cyclosporine or AH. In conclusion, tofacitinib appears to downregulate inflammatory phenomena associated with mast cells and might be a new therapeutic option for patients with refractory CU or UV.

Elevated Serum Levels of Interleukin-15 in Pemphigus Vulgaris Patients: a Potential Therapeutic Target.

Introduction: Pemphigus vulgaris (PV) is a rare autoimmune disease that causes painful blistering. Interleukin-15 (IL-15) as a member of the immunoregulatory cytokines family is associated with the development of the chronic inflammatory or autoimmune disease. There is not much information available in the literature on the exact role IL-15 plays in PV. Objectives: The goal of this study was to evaluate the serum levels of IL-15 in patients with PV and assess the association of IL-15 with anti-desmoglein antibodies and the severity of the disease. Methods: Fifty-three individuals affected with active PV and 38 age- and gender-matched healthy controls were participated in this study. Disease severity was assessed using Autoimmune Bullous Skin Disorder Intensity Score (ABSIS). Serum levels of IL-15 (pg/mL) and anti-desmoglein antibodies (Dsg1, 3) were determined. Results: In the patient group, IL-15 serum levels were statistically higher than those in the control group (3.71 ± 1.5 vs. 0.79 ± 1.03, P < 0.001). A positive correlation was found between serum levels of IL-15 and ABSIS (r = 0.5, P = 0.04). We found no significant correlation between serum concentrations of IL-15 and antidesmoglein antibodies (Dsg1 or Dsg3). Conclusions: An increase in serum level of IL-15 in patients with PV and its relationship with disease severity suggest that this cytokine possibly contributes to the pathogenesis of the disease and targeting IL-15 will likely provide a new insight into the treatment of this disease.

Can Immunofluorescence on Skin/Mucosal Scraping Smear for Pemphigus Diagnosis Substitute Direct Immunofluorescence on Skin Biopsy?

Introduction: Few studies have been conducted on the use of Direct Immunofluorescence (DIF) on skin/mucosal scraping smear for diagnosis of pemphigus disease; however, the diagnostic value of DIF on the smear has not been fully evaluated. Objectives: The present study was carried out to assess the sensitivity and specificity of DIF on skin/mucosal smear for diagnose of pemphigus in the patients presenting with mucocutaneous erosive lesions. Methodology: A total of 89 patients including 40 males and 49 females aged between 23 and 80 years old with various bullous disorders were enrolled in the study. For definite diagnosis, all the patients were subjected to lesional biopsy for pathological studies and perilesional biopsy for DIF studies. In all the cases, skin/mucosal scraping smears were prepared from the perilesional healthy skin/mucosa and were stained with immunofluorescence conjugated anti-IgG. Results: Of 89 patients, 56 (63%) patients were diagnosed with pemphigus. Immunodeposits favoring the pemphigus were demonstrated in the 46 smears of 56 cases of pemphigus (sensitivity of 82%). No case with other types of bullous disease had positive DIF on the smear (specificity of 100%). Conclusion: The findings of the study showed that the sensitivity of DIF on the smear is not high enough to allow us replacing the conventional DIF with smear-DIF for diagnosis of pemphigus, while the specificity of 100% would allow the unequivocal identification of a subset of patients with pemphigus.

Hypertrophic lichen planus on lip mimicking SCC.

The lichen planus (LP) is an inflammatory and immune-mediated disorder that can affect the hair, mucous membranes, nails, and skin. Lichen planus rarely affects the lips. In cases of lip involvement, it presents as radiated streaks, lace-like papules, and erosions. There is no report of lip LP presenting as hypertrophic plaque. Here, we report interesting and rare clinical presentations of LP in a 45-year-old male patient that presented with a verrucous hyperkeratotic plaque on the lower lip mimicking squamous cell carcinoma. However, oral examination revealed, reticulated white patches on the bilateral buccal mucosa, and a biopsy of the lip lesion revealed lichenoid dermatitis which led to the diagnosis of hypertrophic lichen planus. Familiarity with the different clinical presentations of LP and its variants is essential for prompt diagnosis and effective treatment.

Mutation update: The spectra of PLEC sequence variants and related plectinopathies.

Plectin, encoded by PLEC, is a cytoskeletal linker of intermediate filaments expressed in many cell types. Plectin consists of three main domains that determine its functionality: the N-terminal domain, the Rod domain, and the C-terminal domain. Molecular defects of PLEC correlating with the functional aspects lead to a group of rare heritable disorders, plectinopathies. These multisystem disorders include an autosomal dominant form of epidermolysis bullosa simplex (EBS-Ogna), limb-girdle muscular dystrophy (LGMD), aplasia cutis congenita (ACC), and an autosomal recessive form of EBS, which may associate with muscular dystrophy (EBS-MD), pyloric atresia (EBS-PA), and/or congenital myasthenic syndrome (EBS-MyS). In this study, genotyping of over 600 Iranian patients with epidermolysis bullosa by next-generation sequencing identified 15 patients with disease-causing PLEC variants. This mutation update analyzes the clinical spectrum of PLEC in our cohort and in the literature and demonstrates the relationship between PLEC genotype and phenotypic manifestations. This study has integrated our seven novel PLEC variants and phenotypic findings with previously published data totaling 116 variants to provide the most complete overview of pathogenic PLEC variants and related disorders.

Intralesional bleomycin versus intralesional triamcinolone in the treatment of keloids and hypertrophic scars.

Bleomycin is a known chemotherapeutic agent whose beneficial effects have been recently shown in the treatment of keloids and hypertrophic scars, however, it is unclear how effective it is in comparison with corticosteroids. We aimed to compare the safety and efficacy of intralesional bleomycin versus intralesional triamcinolone in the treatment of hypertrophic scars and keloids. Sixty patients were divided into two groups and treated by intralesional injection of triamcinolone (20 mg/ml) or bleomycin (1.5 mg/ml). The treatments were repeated every 3 weeks until the lesions flattened or for a maximum of six sessions. The clinical improvement was evaluated using the Japan scar workshop (JSW) scar scale (JSS) and the physician global assessment of flattening of the lesions. Side effects were also noted and recorded. 55 patients completed the study, 4 patients from the bleomycin group and 1 patient from the triamcinolone group dropped out of the study. In both groups, the total JSS scores decreased significantly after treatment compared to baseline (p < 0.001); however, the difference between groups was not statistically significant after treatment (p = 0.052). Moreover, the degree of flattening of the lesions was comparable between groups (p = 0.933). Side effects in the triamcinolone group were Hypopigmentation(55.2%), atrophy(51.7%), and telangiectasia(41.4%) and in bleomycin group included persistent pain after injection (61.5%), ulceration (69.2%), hyperpigmentation(76.9%), and secondary infection (34.6%). Intralesional bleomycin (1.5 mg/ml) is effective as triamcinolone(20 mg/ml) in the treatment of keloids and hypertrophic scars, however, bleomycin should be used carefully, due to adverse events such as pain, ulceration, and hyperpigmentation.

A rare new presentation of pemphigus vulgaris.

We report a new presentation for pemphigus vulgaris in a 51-year-old female patient that was complaining only from non-healing foot ulcer, but unfortunately pemphigus was not confirmed and the patient lost multiple nails. This new variation is reported to increase health workers' awareness especially in races pemphigus seems to be common.

A review of reported infectious events following rituximab therapy in pemphigus patients.

Pemphigus is a rare autoimmune blistering condition that used to be fatal before the introduction of corticosteroid (CS) and immunosuppressive agents. Rituximab is a monoclonal anti-CD-20 antibody that induces the pathologic B-cells apoptosis with significant efficacy in the treatment of pemphigus. The application of rituximab can lead to infectious events. We aim to review the reported infectious events in pemphigus patients who previously received rituximab and classify them based on the causative agents. A thorough search of PubMed was conducted using the keywords "rituximab," "pemphigus," "infection," "viral disease," "viral infection," "complication," "efficacy" and their combinations also applying their equivalent Mesh terms and including the references cited in each study. All studies that mentioned at least one infectious event were included. A total of 77 infectious events in 68 patients were reported in the literature out of which the most reported causative agent was viral but the most fatal one found to be bacterial. Although rituximab therapy has shown promising results in controlling pemphigus patients mainly the refractory cases, given possible fatal outcomes, we believe the medical profile of the patients before initiating the therapy warrants careful examination to search for any risk factors or predisposing conditions.

Stevens-Johnson Syndrome due to COVID-19 vaccination.

As public COVID-19 vaccination programs are being implemented, it is possible that more rare and serious adverse effects such as Stevens-Johnson syndrome (SJS) and toxic epidermal necrosis (TEN) may occur.

Metabolomic signature of amino acids in plasma of patients with non-segmental Vitiligo.

INTRODUCTION: Vitiligo pathogenesis is complicated, and several possibilities were suggested. However, it is well-known that the metabolism of pigments plays a significant role in the pathogenicity of the disease. OBJECTIVES: We explored the role of amino acids in vitiligo using targeted metabolomics. METHODS: The amino acid profile was studied in plasma using liquid chromatography. First, 22 amino acids were derivatized and precisely determined. Next, the concentrations of the amino acids and the molar ratios were calculated in 31 patients and 34 healthy individuals. RESULTS: The differential concentrations of amino acids were analyzed and eight amino acids, i.e., cysteine, arginine, lysine, ornithine, proline, glutamic acid, histidine, and glycine were observed differentially. The ratios of cysteine, glutamic acid, and proline increased significantly in Vitiligo patients, whereas arginine, lysine, ornithine, glycine, and histidine decreased significantly compared to healthy individuals. Considering the percentage of skin area, we also showed that glutamic acid significantly has a higher amount in patients with less than 25% involvement compared to others. Finally, cysteine and lysine are considered promising candidates for diagnosing and developing the disorder with high accuracy (0.96). CONCLUSION: The findings are consistent with the previously illustrated mechanism of Vitiligo, such as production deficiency in melanin and an increase in immune activity and oxidative stress. Furthermore, new evidence was provided by using amino acids profile toward the pathogenicity of the disorder.

A comparative study of intralesional bleomycin versus cryotherapy in the treatment of condyloma accuminata.

Bleomycin is an antineoplastic agent, which is used off label for various dermatologic conditions. There are numerous reports on the use of intralesional bleomycin (ILB) for the treatment of common warts. However, reports on the efficacy of bleomycin in the treatment of anogenital warts (AGWs) are still limited. The aim is to compare the efficacy/tolerability and recurrence rates of AGW treatment with ILB versus cryotherapy. In this prospective study, 50 patients with AGWs were assigned either to receive triple freeze-thaw cycle of cryotherapy or to receive 1.5 mg/mL ILB for a maximum of four sessions with 3-week intervals. Clinical efficacy was determined by the percentage of the patients with complete clearance. The patients with complete clearance were visited by passing 3 months from the last treatment session to evaluate any recurrence. Of 44 patients completing the study, 16 of 21 (76.19%) patients in the ILB group and 15 of 23 (65.22%) patients in the cryotherapy group showed complete resolution (p value = .425). Moreover, recurrence occurred after 3 months in 18.75% and 46.66% of the ILB and the cryotherapy groups' patients, respectively (p value = .096). The most common local adverse events in both treatment groups were pain, dyspigmentation, and ulceration/erosion, while the delayed ulceration and secondary infection were only observed in the bleomycin group. Intralesional bleomycin is as effective as cryotherapy in the treatment of AGWs, but it is more invasive and associated with post-treatment pain, the delayed ulceration, and cutaneous infection. Intralesional bleomycin is not accompanied with the major risk of necrosis or fibrosis, so the use of ILB in the anogenital area is likely to be safe.This clinical trial was registered in Iranian Registry of Clinical Trials site with code: IRCT20190519043631N1.

Whole-Transcriptome Analysis by RNA Sequencing for Genetic Diagnosis of Mendelian Skin Disorders in the Context of Consanguinity.

BACKGROUND: Among the approximately 8000 Mendelian disorders, >1000 have cutaneous manifestations. In many of these conditions, the underlying mutated genes have been identified by DNA-based techniques which, however, can overlook certain types of mutations, such as exonic-synonymous and deep-intronic sequence variants. Whole-transcriptome sequencing by RNA sequencing (RNA-seq) can identify such mutations and provide information about their consequences. METHODS: We analyzed the whole transcriptome of 40 families with different types of Mendelian skin disorders with extensive genetic heterogeneity. The RNA-seq data were examined for variant detection and prioritization, pathogenicity confirmation, RNA expression profiling, and genome-wide homozygosity mapping in the case of consanguineous families. Among the families examined, RNA-seq was able to provide information complementary to DNA-based analyses for exonic and intronic sequence variants with aberrant splicing. In addition, we tested the possibility of using RNA-seq as the first-tier strategy for unbiased genome-wide mutation screening without information from DNA analysis. RESULTS: We found pathogenic mutations in 35 families (88%) with RNA-seq in combination with other next-generation sequencing methods, and we successfully prioritized variants and found the culprit genes. In addition, as a novel concept, we propose a pipeline that increases the yield of variant calling from RNA-seq by concurrent use of genome and transcriptome references in parallel. CONCLUSIONS: Our results suggest that "clinical RNA-seq" could serve as a primary approach for mutation detection in inherited diseases, particularly in consanguineous families, provided that tissues and cells expressing the relevant genes are available for analysis.

Cytomegalovirus-induced cutaneous ulcer mimicking vasculitis in a patient with systemic lupus erythematous: A case report and review of the literature.

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease which can be complicated with cytomegalovirus (CMV) infection during its course. CMV reactivation can mimic an SLE flare and lead to delay in diagnosis. Here, we reported a previously diagnosed SLE patient who presented with fever, leukopenia, and cutaneous ulcers. Initially, this was diagnosed as an SLE flare and the patient was treated with higher doses of corticosteroids but no improvement was observed. Both nuclear and cytoplasmic inclusions inside the endothelial cells in the skin biopsy as well as positive immunohistochemistry (IHC) staining for CMV antigen were clues to the correct diagnosis of CMV reactivation. Treatment with ganciclovir resulted in clinical resolution. In this report, a very rare clinical form of CMV infection manifesting as cutaneous necrotizing vasculitis on the lower extremity is described and the literature regarding this case is reviewed.