Clinical efficacy of mychophenolate mofetil in treating lichen planopilaris.

Lichen planopilaris (LPP) is a chronic inflammatory disorder of hair without a proven effective and safe treatment. To objectively assess the clinical efficacy of mycophenolate mofetil (MMF) in patients suffering from LPP, a retrospective cohort study was conducted on 52 patients who treated with MMF (2 g/day) at least for 6 months. LPP activity index (LPPAI) before and after treatment was calculated and compared. Most of the patients were female and belonged to the age group of 50-60 years. All of the disease activity indices were significantly improved after 6 months of therapy (p < 0.001). The majority of patients had LPPAI 4-6 and 0-2, before and after treatment, respectively. After 6 months of treatment, half of patients showed a disease activity decrease (LPPAI reduced >25% compared to the baseline value). Systemic MMF is an effective and relatively safe treatment modality for patients with LPP and could lead to significant reduction in disease activity regarding both subjective and objective indices.

Pemphigus patients with initial negative levels of anti-desmoglein: A subtype with different profile?

Pemphigus is a blistering autoimmune disease that is characterized by autoantibodies against desmogleins (Dsg), including anti-Dsg1 and anti-Dsg3. Despite the diagnosis of diseases, the anti-Dsg test by enzyme-linked immunosorbent assay (ELISA) is negative in a small group of pemphigus patients. The aim of this study was to evaluate the clinical course, clinical symptoms, and response to treatment in pemphigus patients with negative levels of anti-Dsg1 and anti-Dsg3. In this study, the data of pemphigus patients referred to Razi Hospital were retrospectively collected from the medical records from 2016 to 2020. Eight patients, whose initial anti-Dsg1/anti-Dsg3 was negative by the ELISA test, were enrolled and their clinical course, clinical signs, and response to treatment were evaluated. The mean age of the subjects (8 females) was 38.75 ± 12.09. The most common phenotype of the subjects was pemphigus vulgaris (PV) with mucosal involvement. Additionally, the common site of blister inception was mouth of the patients. The mean prednisolone dose received by the patients at the initiation was 32.5 ± 13.62 mg/day. According to Pemphigus disease area index (PDAI), six patients had mild severity, while two cases had moderate severity. Among the patients, six subjects received rituximab (RTX). Also, five patients experienced remission after 6.2 ± 5.21 months. PV is the most common phenotype of the disease and mucosal involvement is more common in patients with negative anti-Dsg-1/3 results. The severity of the lesions in most of the patients is mild at baseline and most patients seems to respond to RTX therapy and reach remission.

Overestimation of subclinical atherosclerosis in psoriasis.

This study is one of a few that have been conducted to evaluate subclinical atherosclerosis in Iranian patients with psoriasis. Based on our findings, routine ultrasonography evaluation of all of patients with psoriasis does not seem to be reasonable or cost-effective.

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.

Oral simvastatin combined with narrowband UVB for the treatment of psoriasis: A randomized controlled trial.

Psoriasis is a common chronic skin condition, which is an immune-related hyperproliferative disorder. Among the different treatments for psoriasis, statins have been found to reduce the severity of the disease. Accordingly, fluvastatin and simvastatin are known to have anti-inflammatory effects by inhibiting inflammatory cytokines and lymphocyte function. Narrowband ultraviolet B (NB-UVB) is known as an effective and safe modality for psoriasis treatment. In this double blind, randomized controlled trial, we investigated the efficacy and safety of adding simvastatin to NB-UVB phototherapy in patients with psoriasis. Forty-eight patients with psoriasis undergoing NB-UVB phototherapy were randomly divided into placebo groups; one received oral simvastatin, and the other received a placebo for 12 weeks. Psoriasis severity was assessed with the Psoriasis Area and Severity Index (PASI) and Dermatology Life and Quality Index (DLQI). Both groups showed a significant decline in PASI score after 6 and 12 weeks compared to the baseline. The differences in reducing PASI score and DLQI between the two groups were not significant neither at week sixth nor 12th. In addition, DLQI decreased significantly in the placebo group at week 12th. In contrast with previous studies, we did not find any additional effects for oral simvastatin5 in treating psoriasis with NB-UVB. Also, an insignificant difference in the improvement of quality of life between both groups was ascertained.

Adverse electrocardiographic effects of rituximab infusion in pemphigus patients.

Pemphigus encompasses a group of chronic autoimmune blistering diseases of the skin and/or mucosa. Rituximab (RTX) has shown promising efficacy for the treatment of pemphigus in the past decade. Considering potential cardiac side effects, this study was conducted to assess the effects of RTX on electrocardiogram (ECG) parameters in pemphigus patients. This observational cross-sectional study was conducted in 80 consecutive patients with pemphigus who were eligible for RTX infusion. The patients' heart rhythm was monitored before, during and after RTX infusion and ECG parameters were compared before and after the infusion. Eighty patients were included in the study. The median age of the patients was 42 years. The mean and maximum heart rate (HR) increased significantly after RTX infusion compared with pre-infusion mean and maximum HR. The mean corrected QT (QTc) interval, premature atrial contraction (PAC), and premature ventricular contraction (PVC) counts increased significantly after RTX infusion (P value: 0.009, 0.004 and 0.007 respectively). According to the results of this study, RTX has potential arrhythmogenic side effects including increased mean and maximum HR, QTc interval, PAC and PVC count. However, these findings are minor and should not prevent eligible patients from receiving RTX infusion.

Decreased serum levels of interleukin-17, interleukin-23, TGF-ß in pemphigus vulgaris patients, and their association with disease phase.

The exact pathogenesis of Pemphigus Vulgaris (PV) has remained unclear, but it seems that cytokines play critical roles in this disease. This study aims to assess the serum levels of interleukin (IL)-6, IL-17, IL-23, and TGF-ß in PV patients and compare the results to the healthy controls. Serum levels of IL6, IL-17, IL-23, and TGF-ß were successfully determined by enzyme-linked immunosorbent assay (ELISA) in 27 newly diagnosed PV, 32 patients in remission, and 29 healthy controls. It was shown that the mean serum levels of IL-17, IL-23, and TGF-ß serum are significantly different among the PV patients and healthy controls (P values: <.001, .001, and .003, respectively). It was found that new PV patients have lower serum levels of IL-17, IL-23, and TGF-ß as compared to healthy controls (P values: <.001, <.001, and .003, respectively). Regarding IL-6, no significant difference was observed between the healthy controls and the other two groups of patients. IL-17, IL-23, and TGF-ß are involved in the pathogenesis of PV. However, more studies are required to clarify their exact roles in the immunopathogenesis of PV.

Inherited Interleukin 2-Inducible T-Cell (ITK) Kinase Deficiency in Siblings With Epidermodysplasia Verruciformis and Hodgkin Lymphoma.

Biallelic mutations in the ITK gene cause a T-cell primary immunodeficiency with Epstein-Barr virus (EBV)-lymphoproliferative disorders. We describe a novel association of a homozygous ITK mutation with ß-human papillomavirus (HPV)-positive epidermodysplasia verruciformis. Thus, loss of function in ITK can result in broad dysregulation of T-cell responses to oncogenic viruses, including ß-HPV and EBV.

Iranian guideline for rituximab therapy in pemphigus patients.

Pemphigus vulgaris (PV) is an autoimmune blistering disease affecting the skin and/or mucosa. Rituximab (RTX) has been approved recently by US FDA as an effective and safe treatment of PV. The high incidence of PV in Iran encouraged our team to prepare a consensus guideline for RTX administration based on literature review and a decade experience of an expert panel. RTX is recommended for the treatment of new cases of PV as well as patients not responding to conventional therapy. Contraindications include history of anaphylaxis or IgE-mediated hypersensitivity to murine proteins of RTX, severe active infections, pregnancy, breastfeeding, severe heart failure, and arrhythmia. Prophylactic antiviral therapy is recommended in patients at risk of reactivation of HBV and isoniazid for those at risk of reactivation of tuberculosis. Concomitant use of systemic corticosteroids is recommended as a rule. Except for methotrexate, the combination with other immunosuppressive drugs is discouraged. Intravenous immunoglobulin is recommended for those at risk of infections or with extensive disease. The recommended dosage of RTX for the first cycle is 2 g either 500 mg weekly or 1 g biweekly. There is no general consensus whether the next doses of RTX be administered upon relapse or as maintenance therapy. We strongly recommend RTX sooner in the course of pemphigus.

Acantholytic and Pagetoid Variant of Bowen's Disease with Microinvasion on the Scalp of a Young Female Patient: A Case Report.

Bowen's disease (BD) is a non-melanoma skin cancer with several histological subtypes. Herein we describe a case of a 35-year-old woman with a 4-cm diameter crusted plaque on the parietal scalp region. She had the lesion for 2 years. It had previously been histologically diagnosed as pemphigus vulgaris and only treated with a topical cream. The lesion progressively became thicker and larger. A new biopsy showed atypical cell proliferation through the whole thickness of the epidermis and follicular epithelium, with dermal microinvasion along with acantholysis and clear cell formation. The patient underwent total lesion excision (1 cm margin) with the diagnosis of both acantholytic and pagetoid subtypes of BD and dermal microinvasion. We describe a rare case of a young female patient with both subtypes of BD present in one lesion on an area not exposed to the sunlight. The lesion was initially misdiagnosed as pemphigus vulgaris.

Cheilitis in acne vulgaris patients with no previous use of systemic retinoid products.

BACKGROUND/OBJECTIVES: Isotretinoin is commonly used in the treatment of acne vulgaris. While one of the more common side-effects is cheilitis, we have observed an increased incidence of cheilitis prior to the commencement of systemic isotretinoin. The aim of this study was to assess the prevalence of cheilitis among acne vulgaris patients. METHODS: A non-interventional cross-sectional study of patients with acne vulgaris. Patients with previous use of systemic retinoids were excluded. The patients were examined for signs and symptoms of cheilitis. RESULTS: Of a total of 400 patients, 134 (34%) had evidence of cheilitis at initial presentation. Two-thirds (63%) were female (P < 0.001). The distribution of the cheilitis was as follows; 55% on the lower lip, 30% on both lips, and 16% on the upper lip. Over a quarter (27%) of patients with cheilitis had acne excorie, compared with only 8% of patients with no signs of cheilitis. CONCLUSION: Our findings suggest that cheilitis is quite common among acne vulgaris patients even before treatment with isotretinoin.

Characteristic features of cutaneous melanoma in a dermatology referral centre in Tehran, Iran.

BACKGROUND/OBJECTIVES: The characteristics of cutaneous melanoma in the Middle-Eastern countries is poorly described. Therefore we conducted this study to determine the characteristics of melanoma in Iran. METHODS: A retrospective, cross sectional study of melanoma patients seen at a tertiary referral centre, Iran, from May 2004 to October 2014. Clinical data included age and gender of the patients at the time of diagnosis, tumour location and tumour size. Histological characteristics included Breslow thickness, Clark level and subtype of tumour. RESULTS: A total of 450 cases of melanoma with a male/female ratio of 1.1:1 were reviewed. The mean age of patients was 57.5 years. The most frequent histological subtypes were acral lentiginous melanoma (30%) and lentigo maligna melanoma (29%). In 215 cases (49%) the tumour was located on the extremities. The second most common site was the face. Tumour invasion was mainly at Clark level III and IV. The mean Breslow thickness was 2.8 mm; 143 (38%) melanomas had a Breslow thickness less than 1 mm (T1) and 86 (23%) were more than 4 mm (T4). CONCLUSION: This study indicates that clinical and histological features of melanoma in Iranians (who are mainly of skin phototypes 3-4) are different from those observed in Western countries. Further cohort studies are required to evaluate the role of ethnic and environmental risk factors for melanoma in different populations.

Psoriasiform mycosis fungoides: a rare form of the disease with review of the literature.

BACKGROUND: Mycosis fungoides (MF) typically presents as erythematous scaly patches or plaques that may progress to cutaneous tumors. Although MF may be presented like other dermatoses, initial presentation as psoriasiform plaques simulating psoriasis is rare. Differentiating MF from psoriasis is important because systemic therapies used for psoriasis can worsen MF. We describe a case of psoriasiform MF and we also review the clinicopathological features of similar cases in the literature. CASE: A 46-year-old woman was referred to our clinic with a history of psoriasiform plaques for 13 years. She had multiple, generalized, indurated plaques with thick psoriasiform scales that were unresponsive to topical treatments. The histopathology showed marked psoriasiform epidermal hyperplasia with epidermotropic atypical lymphocytes compatible with MF. Immunohistochemical (IHC) staining showed that atypical lymphocytes were positive for CD3, CD4, CD8, and CD5. Of note, upper dermal and intraepidermal large atypical lymphocytes were CD30 positive. The review of similar psoriasiform MF cases revealed that they had all been treated as psoriasis for many years and finally diagnosed as MF especially after deterioration induced by immunosuppressive therapies. CONCLUSIONS: In presumed cases of psoriasis that are unresponsive to treatment, progressive, or ulcerative, biopsy should be considered to rule out MF, particularly before starting a potent immunosuppressive agent.

Oxidative stress and antioxidant markers in patients with alopecia areata: A comparative cross-sectional study.

Background Alopecia areata is a chronic inflammatory skin disease. Oxidative stress may contribute to the pathogenesis of this condition. Aim To evaluate the serum oxidative stress markers and antioxidant capacity in patients with alopecia areata. Methods This cross-sectional study was performed on 40 patients with alopecia areata and 40 healthy controls. The fasting blood sugar, C-reactive protein, lipid profile, and serum oxidative markers, including advanced glycation end products and advanced oxidation protein products, were measured in this study. Also, antioxidant enzymes, including paraoxonase-1, lecithin-cholesterol acyltransferase and serum ferric-reducing antioxidant power, were determined. Results The serum levels of advanced glycation end products and advanced oxidation protein products were significantly higher in patients with alopecia areata, compared to the controls (P < 0.001), whereas the levels of ferric-reducing antioxidant power, paraoxonase-1 and lecithin-cholesterol acyltransferase were significantly lower in patients with alopecia areata, compared to the controls (P < 0.001). The mean fasting blood sugar level was significantly higher in patients with alopecia areata, compared to the controls. The ferric reducing antioxidant power level was significantly associated with the percentage of hair loss (P = 0.01, r = 0.4) and the serum C-reactive protein level (P = 0.03, r = -0.3) in patients with alopecia areata. Limitations Since the current study had a cross-sectional design, no cause-effect relationship was established between alopecia areata and oxidative stress. The sample size of our study was also small. Conclusion Based on the present results, the oxidant-antioxidant enzymatic system is impaired in alopecia areata due to the increased oxidative products and decreased antioxidant activity.

Combination therapy for management of pemphigus patients with unexpected therapeutic response to rituximab: A report of five cases.

Key Clinical Message: The immunosuppressant agents should be considered earlier in the course of treatment with rituximab, possibly after the unfavorable response at first cycle of treatment, especially in male patients and those with high BMI. Abstract: Rituximab (RTX) has recently been proposed as an alternative first-line therapy for pemphigus patients. However, there are some rare reports of worsening of pemphigus following RTX therapy in the literature. This study aimed to evaluate the efficacy and safety of using a combination treatment of mycophenolate mofetil or dapsone and methotrexate in case of nonresponse, exacerbation or development of allergic reactions following rituximab therapy in pemphigus patients. In this case series, archive files of pemphigus patient in a tertiary care hospital from 2016 to 2021 who were treated with rituximab were reviewed and those with failure in treatment process including nonresponsiveness, exacerbation or development of allergic reactions to rituximab were identified and assessed. The study includes five patients out of 1245 RTX-treated patients, who did not respond to RTX (one patient) or experienced an exacerbation of disease (two patients) or development of allergic reactions (two patients). Male patients with high BMI (BMI > 25) whose response to rituximab was not good at first cycle and happened to receive rituximab later in the course of disease, had highest number of relapses and benefited the most from this combination immunosuppressive treatment as an alternative for repeating rituximab cycles. The lower risk of relapse and a better chance of remission might indicate the efficacy of adjuvant immunosuppressant therapy in patients with no-response, exacerbation, or allergic reaction to rituximab. These therapeutic effects were better observed in patients who received lower doses of rituximab which could suggest that the immunosuppressant agents should be considered earlier in the course of the disease, possibly after the first failed trial of rituximab therapy.

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.

Metabolic derangement in patients with vitiligo: a cross-sectional study.

Vitiligo is a pigmentary disorder resulting from progressive destruction of melanocytes in the skin. There is a growing body of evidence about higher risk of metabolic syndrome and dyslipidemia in some dermatoses including vitiligo. We aimed to evaluate lipid profile, leptin and C reactive protein (CRP) status among Iranian patients with vitiligo, compared with healthy controls and perused the relationship between abnormal values of these parameters with disease duration and physical characteristics of patients. 40 patients with vitiligo and 40 age-matched and sex-matched healthy controls were enrolled in the study. Data on weight, height, lipid profile, leptin and CRP values were recorded and compared. The mean values for nearly all study parameters (except for high-density lipoprotein) were significantly higher in patients with vitiligo, compared with healthy controls, irrespective of age and sex. We could not find any correlation between vitiligo and study parameters, regarding disease severity and extension of lesions; but in patients who have been suffering from vitiligo for more than 5 years, systolic blood pressure, diastolic blood pressure and CRP values were noted to be significantly higher (p<0.001, p=0.003 and p=0.03, respectively). In conclusion, screening of patients with vitiligo in regard to their lipid profile as well as blood pressure should be considered, especially in patients with longer disease duration or those who have other cardiovascular risk factors to prevent morbidity and mortality as a result of developing cardiovascular events.