Iranian psoriasis registry: Research protocol and preliminary results of a pilot study.

BACKGROUND: Psoriasis as a common cutaneous inflammatory disease affect many aspects of patients' life. Disease registries render it possible to collect valuable data regarding a disease prevalence and burden as well as long-term observations concerning possible therapeutic regimens. METHODS: This registry was designed for the ongoing systematic data collection on patients with psoriasis at two referral dermatology centers in Iran. The pilot phase of the registry was used to identify possible obstacles in the application and execution of systematic registration. RESULTS: A total of 281 patients were registered with the mean age of 42.02 years. The disease duration was 12.06 ± 10.90 years with the variety of clinical presentations. There was no significant difference between males and females in the age of disease onset (p = 0.53). Notably, 167 patients had children. Among them, 13 had children with psoriasis. The gender of the affected parent did not affect the possibility of psoriasis transmission to the child, and no significant difference was seen between the two sexes (P = 0.569). Regarding treatment, 99.4% of patients (n = 280) had used topical agents, 52.3% (n = 147) biologics, and 60.9% (n = 171) nonbiologic medications. CONCLUSION: Clinical trials report the efficacy and safety data regarding limited study populations in a restricted time window, and the results may differ from the general population. This highlights the importance of registry-based studies for collecting and analyzing longitudinal information. In terms of long-term disease complications such as malignancies, cardiovascular events, and serious adverse events, registry-based studies will help clinicians better recognize and manage each disease.

Facial pyoderma gangrenosum associated with fallopian tube carcinosarcoma.

Pyoderma gangrenosum (PG) is a neutrophilic dermatosis associated with underlying disorders. The association between PG and solid organ tumors (SM), including gynecologic cancers, has been previously reported. Here, we report a case of a 61-year-old woman with pyoderma gangrenosum on the posterior auricular region associated with an underlying fallopian tube carcinosarcoma: a rare and aggressive gynecologic malignancy. The patient's ulcer responded favorably to treatment, and surgical resection of the tumor was performed. The patient was then referred for further cancer management. No new lesions or recurrences were found over the 18 months of routine follow-up.

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.

Progressive mucinous histiocytosis treated successfully with thalidomide: a rare case report.

Hereditary progressive mucinous histiocytosis (HPMH) is an extremely rare progressive non-Langerhans cell histiocytic disorder presenting with only cutaneous manifestations. Patients typically present with multiple asymptomatic dome-shaped erythematous papules, usually involving the face and upper extremities. Twenty-six cases have been reported worldwide, with no spontaneous regression. Treatment with thalidomide stopped the progression of the disease in two cases. We report a case of progressive mucinous histiocytosis in a 31-year-old female patient with a history of tuberculosis who presented papular lesions on the face that later extended to the hands. She was treated with isoniazid for tuberculosis and isotretinoin for the skin lesions; the improvement was minimal during the next two months, with new lesions appearing on both hands. Thalidomide stopped the progression of the disease. The cause and pathogenesis of HPMH are undetermined. The pathogenesis of HPMH may be similar to that of lysosomal storage disease, considering the intra-cytoplasmic phospholipid deposition in both diseases, in addition to the likelihood of a role of macrophages in triggering the disease. In our patient, tuberculosis may have contributed.

The emerging role of T cells in pemphigus vulgaris: a systematic review.

Pemphigus vulgaris is a potential life-threatening autoimmune bullous disorder. The significant role of autoreactive B cells in the pathogenesis of PV has been explained extensively by producing autoantibodies. Recently, attention has been directed toward the role of T cells in the pathogenesis of PV; in other words, the underlying etiology of PV depends on the interaction between T cells and B cells resulting in antibody secretion. Herein, we systematically review the current literature on the emerging role of T cells in PV. To perform this systematic review, an extensive search through EMBASE, PubMed, Scopus, and ISI databases was performed from 1976 through 2021. Articles investigating the function of T cell subgroups in the pathogenesis or treatment of pemphigus vulgaris were included and reviewed. It is evidenced that T cells play a pivotal role in PV pathogenesis. Th1 and Th2 dichotomy including Th1 suppression and Th2 elevation may induce antibody production against desmoglein in keratinocytes. Furthermore, increased level of Th17 and decreased level of regulatory T cells have been detected in PV patients. However, further studies on the exact role of γδ-T cells in PV are required in order to clarify the pathogenesis of PV. T cells and their subtypes can be involved in the pathogenesis of PV. Thus, they can be considered as tentative targets of novel therapies for PV.

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.

The presence of mast cells in lichen planopilaris and discoid lupus erythematosus of the scalp: A quantitative study.

BACKGROUND: Histopathologic differentiation of lichen planopilaris (LPP) and discoid lupus erythematosus (DLE) as two common causes of primary cicatricial alopecias remains challenging. METHOD: We performed a histopathologic study on a case series of LPP and DLE specimens to investigate the number, distribution, and morphology of mast cells as indices for differentiation of these two entities. H&E investigation and Giemsa staining for the detection of mast cells was performed. RESULT: A total of 74 cases comprising 50 cases of LPP and 24 cases of DLE were assessed. The mean mast cell count and percentage were significantly higher in LPP group (p < 0.001). Mean degranulated mast cell count and the mean intact mast cell count were also significantly higher in LPP patients (p < 0.001). Most of the specimens, 58 (78.4%), showed both perifollicular and perivascular distribution of mast cells without significant difference between two groups. The morphology of mast cells was predominantly round-oval in 85.5%, predominantly fusiform in 13.5% with more frequent fusiform morphology in DLE group. CONCLUSION: The mast cell count detected by Giemsa staining could assist pathologists in distinguishing between LPP and DLE.

Evaluation of anxiety and depression in patients with morphea taking immunosuppressive drugs during the COVID-19 pandemic.

BACKGROUND: During the coronavirus disease 2019 (COVID-19) pandemic, individuals especially those with chronic diseases face various problems like psychological distress. AIM: To evaluate anxiety and depression among morphea patients taking immunosuppressants compared with controls during the COVID-19 pandemic. METHODS: In this case-control study, electronic data of 42 eligible morphea patients and 42 age- and sex-matched healthy subjects were extracted. All participants were asked about adherence to the health protocols and the symptoms of COVID-19. To investigate anxiety and depression, we provided the participants with an online version of the Hospital Anxiety and Depression Scale (HADS) Questionnaire. In this standard questionnaire, a score ≥ 11 represents a significant psychological disorder. RESULTS: The two groups did not differ significantly in the mean HADS-Anxiety (HADS-A) and HADS-Depression (HADS-D) scores (p= .08 and p= .79, respectively). HADS-A and HADS-D scores were ≥ 11 in 16.67% and 11.90% of the patients, respectively. Among the morphea patients, those with sleeping problems during COVID-19 had a significantly higher mean HADS-D score (p= .048). Notably, 21.43% of patients discontinued their treatment due to fear of COVID-19 and experienced disease recurrence. Adherence to health protocols was higher in patients compared with controls. The rate of COVID-19 occurrence was not significantly different between the groups. CONCLUSIONS: Although there was no significant difference in anxiety and depression between the groups, it is recommended to provide psychiatric counseling opportunities to morphea patients to improve treatment outcomes during this pandemic.

The role of interleukin-17 (IL-17) in the pathogenesis of discoid lupus erythematosus and lichen planopilaris: is immunohistochemistry for IL-17 a promising way to differentiate these entities?

BACKGROUND: Interleukin-17 (IL-17) is a pro-inflammatory mediator which its excess secretion by immune cells is found in many chronic autoimmune diseases. This survey is aimed to compare the amount and pattern of distribution of IL-17 positive cells in lichen planopilaris (LPP) and discoid lupus erythematosus (DLE) which are of the most frequent causes of primary cicatricial alopecia (CA). METHODS: Biopsies of lesional scalp skin from adult LPP (n = 30), DLE patients (n = 19), and control scalp skin (n = 18) were analyzed by immunohistochemistry (IHC) method using rabbit polyclonal antibody against IL-17. Cells stained positive for IL-17 and their pattern of distribution were assessed and compared between these groups. RESULTS: The mean of IL-17 positive cell counts per high power field (HPF) in both LPP and DLE groups was significantly higher in comparison with the control group (both p < .0001). The LPP group had higher values of IL-17 positive cell counts per HPF (47.56 ± 13.37) compared to the DLE group (22.21 ± 11.06) (p < .0001). More than 30 IL-17 positive cell counts in IHC have a sensitivity of 90% and a specificity of 78% for differentiating LPP from DLE. CONCLUSIONS: Our findings suggest that IL-17 is effective in the pathogenesis of DLE and LPP and its role is more prominent in LPP. IL-17 positive cells in IHC can distinguish DLE from LPP with satisfactory sensitivity and specificity.

Soluble biomarkers for diagnosis, monitoring, and therapeutic response assessment in psoriasis.

BACKGROUND: Psoriasis is an inflammatory disease associated with multiple comorbidities. Biomarkers for the assessment of psoriasis, its associated comorbidities, and the therapeutic response are not well characterized. A number of possible biomarkers for the diagnosis and monitoring of psoriasis have been proposed. PURPOSE: To assess potential biomarkers for diagnosis of psoriasis, its associated comorbidities and response to treatment. METHODS: We investigated medical databases from 2000 to 2021 and assessed relevant research. In this review, we evaluated the important biomarkers to help predict potential risk of psoriasis and disease activity (Beta-defensin-2, VEGF, Lipocalin-2, and YKL-40) and its possible inflammatory-related comorbidities like cardiovascular diseases (hs-CRP, GlycA, Psoriasin, IL-18, NT-proBNP, and Adipokines). In addition, we described the potential biomarkers for psoriatic arthritis (CXCL10, S100A8 and S100A9, and MicroRNA) and related manifestations such as enthesitis. Finally, we discussed novel markers for monitoring the response to specific treatments (HLA-C 06, PLC, TARC, NLR, and PLR) as well as potentially useful biomarkers for evaluation of therapy-associated adverse events (liver fibrosis-related markers). CONCLUSION: A wide range of genetic, tissue, and serum markers have been investigated in psoriasis; however, most of them are not used in routine clinical practice; and thorough physical examination along with the appropriate application of clinical scoring systems like Psoriasis Area and Severity Index score are still of particular importance.

Genital syringocystadenocarcinoma papilliferum: An unusual location and review of the literature.

Syringocystadenocarcinoma papilliferum (SCACP) is an extremely rare adnexal neoplasm of the sweat glands. It is believed to arise from the malignant transformation of syringocystadenoma papilliferum (SCAP). The majority of cases present on the head and neck and up to 17% of cases show metastatic progression. These tumors seldom occur in the anogenital area and, to date, only one case has been reported on the penis. Here, we report a rare case of SCACP in a 65-year-old man who presented with an erythematous, non-healing, ulcerated lesion on the penis.

Cutaneous adverse events related to COVID-19 vaccines: A cross-sectional questionnaire-based study of 867 patients.

Considering the emergency approval of the Food and Drug Administration for widespread use of coronavirus disease 2019 (COVID-19) vaccines, evaluating potential vaccine-related adverse effects is critical as it will allow physicians to diagnose and manage these complications properly. In this descriptive cross-sectional questionnaire-based study, we evaluated the possible side effects of the COVID-19 vaccine from June 1, 2021 to June 21, 2021. The Iranian population is generally vaccinated with AstraZeneca, Sputnik V, Sinopharm, and Bharat vaccines. The continuous and categorical variables were described and data analyzed by the SPSS software version 25. Cutaneous reactions occurred in 30% of individuals vaccinated against COVID-19. The most common cutaneous complications were focal injection site reaction, exanthematous rash, and urticaria. There were infrequent cutaneous adverse events that included vesicular eruption, pernio-like lesions, angioedema, erythema multiforme-like eruption, and zoster. Acquainting physicians with COVID-19 vaccine-related cutaneous complications will assist them in detection and management. In addition, introducing these complications to individuals might improve acceptance of vaccine-related adverse effects in the general population.

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.