Publications by authors named "Maryam Daneshpazhooh"

156 Publications

A systematic review on efficacy, safety, and treatment-durability of low-dose rituximab for the treatment of Pemphigus: special focus on COVID-19 pandemic concerns.

Immunopharmacol Immunotoxicol 2021 Jul 21:1-12. Epub 2021 Jul 21.

Department of Dermatology, Rasool Akram Medical Complex, Iran University of Medical Sciences, Tehran, Iran.

Background: Rituximab is a FDA-approved monoclonal antibody for adults with moderate to severe potentially life-threatening pemphigus vulgaris. Recent studies have focused on assessments of efficacy and safety of low-dose rituximab (<2 gram in each cycle).

Method: Databases were searched from 2010 to 2020 (last update: 1 June 2020).

Result: Nine studies were entered; including180 cases (92: women, 88: men, age range: 9-83 years). The dosages of each Rituximab cycle varied between ultra-low-dose (≤500 mg for a cycle, either multiple infusions or a single infusion), low-dose (2 × 375 mg/m or 2 × 500 mg) and modified-dose (3 × 375 mg/m or 3 × 500 mg). The efficacy and safety of Rituximab in the studies are known by the recovery time, relapse time, and side events. According to the studies, 2 × 500 can lead to complete remission in a broad range, from 35 to 82%. These differences might be explained by different end-points and variable cumulative corticosteroid dosage after RTX administration. Although the studies showed that low dose RTX is efficient, there are some controversies regarding the choosing low-dose for severe patients.

Conclusion: Considering the effectiveness of low-dose, intermediate dose, and ultra-low-dose protocols of Rituximab in inducing remission in pemphigus disease and considering factors such as cost of therapy, and the need to induce a minimum of immunosuppression for a minimum duration in the COVID-19 pandemic, suggested to use low-dose Rituximab protocol (2 infusions of 500 mg Rituximab: interval of 2 weeks) to induce the remission in mild-to-moderate pemphigus patients.
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http://dx.doi.org/10.1080/08923973.2021.1953063DOI Listing
July 2021

International Dermoscopy Society criteria for non-neoplastic dermatoses (general dermatology): validation for skin of color through a Delphi expert consensus.

Int J Dermatol 2021 Jul 2. Epub 2021 Jul 2.

First Department of Dermatology, School of Medicine, Faculty of Health Sciences, Aristotle University, Thessaloniki, Greece.

Background: The International Dermoscopy Society (IDS) recently released a set of five basic dermoscopic parameters (vessels, scales, follicular findings, "other structures," and specific clues) encompassing a total of 31 subitems to standardize the use of dermoscopy in non-neoplastic dermatoses, yet they have been developed taking into account Caucasian/Asian skin, with consequent possible limitations if used in dark skin.

Objectives: To validate the abovementioned criteria for the use in dark-skinned patients (phototypes IV-VI) through an expert consensus.

Methods: The two-round Delphi method was adopted, with an iterative process consisting of two rounds of email questionnaires. Potential panelists were recruited via e-mail from all over the world based on their expertise on dermoscopy of non-neoplastic dermatoses in skin of color.

Results: Twenty-two panelists took part in the validation process. All of the five originally proposed parameters and subitems reached agreement during the first round, aside from "follicular red dots." Additionally, during round 1, five new subitems were proposed (perifollicular scales distribution, follicular openings obliteration, broken hairs, eccrine pigmentation, and eccrine ostia obliteration), along with the possibility to change the denomination of parameter 3 (from "follicular findings" to "follicular/eccrine findings") and split it into two subparameters ("follicular findings" and "eccrine findings"). All such proposals reached agreement during the second round and therefore were included in the final list, for a total of 37 items.

Conclusions: Although nearly all the dermoscopic criteria originally proposed by the IDS are applicable even to darker phototypes, several additional variables need to be assessed.
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http://dx.doi.org/10.1111/ijd.15729DOI Listing
July 2021

Toxic Epidermal Necrolysis in Pregnancy due to Ondansetron with a Favorable Outcome: A Case Report and Review of the Literature.

J Clin Aesthet Dermatol 2021 May 1;14(5):46-49. Epub 2021 May 1.

Drs. Shakoei and Hamzelou are with the Department of Dermatology at Imam Khomeini Hospital, Tehran University of Medical Sciences in Tehran, Iran.

Toxic epidermal necrolysis (TEN) is an uncommon, life-threatening hypersensitivity drug reaction with a high mortality rate that involves the skin and mucous membranes. Most reported cases involving pregnant patients were seen in those with human immunodeficiency virus. Here, we discuss a 21-year-old Iranian woman who presented at 18 weeks' gestation with extensive TEN following the administration of ondansetron with no any other risk factors.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8211332PMC
May 2021

Current status and prospects for the diagnosis of pemphigus vulgaris.

Expert Rev Clin Immunol 2021 Jul 6:1-16. Epub 2021 Jul 6.

Department of Dermatology, Autoimmune Bullous Diseases Research Center, Tehran University of Medical Sciences, Tehran, Iran.

: Pemphigus vulgaris (PV) is an intraepidermal autoimmune bullous disease (AIBD) characterized by autoantibodies against desmosomal adhesion proteins, most commonly desmoglein (Dsg)3, leading to the suprabasal cleft formation and acantholysis.: Direct immunofluorescence (DIF) and indirect immunofluorescence (IIF) studies display the intercellular deposition of IgG/C3 throughout the epidermis and presence of circulating autoantibodies respectively, as a net-like pattern. However, the target antigen remains unknown using immunofluorescence techniques. Thanks to the development of Dsg ELISA, using recombinant technology, circulating antibodies against Dsg1 and 3 could be detected sensitively. It is possible to differentiate PV from pemphigus foliaceus (PF) using this assay. BIOCHIP mosaic and multivariant ELISA are two novel serologic methods with the added value of the ability to screen several AIBDs simultaneously.Non-Dsg1/3 antigens are also involved in the pathogenesis of PV and investigated more deeply thanks to the protein microarrays technique. Additionally, patients with high values of anti-Dsg1/3 may be lesion-free, suggesting the presence of nonpathogenic autoantibodies.: Newer diagnostic methods to replace traditional techniques should possess high sensitivity and specificity and be widely available, noninvasive, and relatively cheap. The newly developed methods need to be further evaluated before being recommended for routine use.
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http://dx.doi.org/10.1080/1744666X.2021.1945925DOI Listing
July 2021

Rituximab exhibits a better safety profile when used as a first line of treatment for pemphigus vulgaris: A retrospective study.

Int Immunopharmacol 2021 Jul 24;96:107755. Epub 2021 May 24.

Department of Epidemiology, The Warren Alpert Medical School of Brown University, Providence, RI, USA.

Background: Pemphigus is an autoimmune disease that is challenging to treat and has few available therapeutic options. Recently, several studies have demonstrated that rituximab may be an efficacious first-line treatment in newest guidelines.

Aim: To compare the side effect profiles of rituximab administered after a course of immunosuppressant agents versus as a first-line therapy and evaluate the impact of patient characteristics and disease severity indices on occurrence of adverse effects.

Methods: A retrospective cross-sectional study was conducted on 999 patients with pemphigus vulgaris who received rituximab either as a first-line treatment or after conventional adjuvant therapies. The occurrence of partial or complete remission as well as the incidence of drug-related adverse effects were evaluated and compared between the two groups.

Results: Smoking, pulmonary comorbidity, and mucocutaneous phenotype were associated with an increased risk of developing infectious complications by 12.49, 5.79, and 2.37 fold, respectively. These associations were more prominent among those who received rituximab after immunosuppressant agents.

Conclusions: Early use of rituximab benefits pemphigus patients, especially those with a mucocutaneous phenotype, pulmonary comorbidity, or history of smoking, and reduces their risk of infectious adverse events.
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http://dx.doi.org/10.1016/j.intimp.2021.107755DOI Listing
July 2021

COVID-19: The experience from Iran.

Clin Dermatol 2021 Jan-Feb;39(1):23-32. Epub 2020 Dec 15.

Department of Dermatology, Tehran University of Medical Sciences, Tehran, Iran; Autoimmune Bullous Diseases Research Center, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran.

The first cases of coronavirus disease 2019 (COVID-19) in Iran were detected on February 19, 2020. Soon the entire country was hit with the virus. Although dermatologists were not immediately the frontline health care workers, all aspects of their practice were drastically affected. Adapting to this unprecedented crisis required urgent appropriate responses. With preventive measures and conserving health care resources being the most essential priorities, dermatologists, as an integral part of the health system, needed to adapt their practices according to the latest guidelines. The spectrum of the challenges encompassed education, teledermatology, lasers, and other dermatologic procedures, as well as management of patients who were immunosuppressed or developed drug reactions and, most importantly, the newly revealed cutaneous signs of COVID-19. These challenges have paved the way for new horizons in dermatology.
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http://dx.doi.org/10.1016/j.clindermatol.2020.12.009DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7836797PMC
May 2021

Decreased Serum Levels of Interleukin-4 and Interleukin-21 in New Pemphigus Vulgaris Patients, but Not Chronic Patients With Inactive Disease Compared to Healthy Controls.

Dermatol Pract Concept 2021 Mar 12;11(2):e2021035. Epub 2021 Apr 12.

Autoimmune Bullous Diseases Research Center, Department of Dermatology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran.

Background: Pemphigus is a rare group of autoimmune blistering diseases with unknown etiology and unclear pathogenesis. Pemphigus vulgaris (PV) is the most common subtype, and is characterized by ulcerations or flaccid blisters on mucous membranes and on the skin. It is accepted that cytokines have a critical role in the pathogenesis of PV, while their exact roles remain to be elucidated.

Objectives: This study assessed serum levels of interleukin (IL)-4 and IL-21 in different phases of the disease in comparison with healthy controls.

Methods: In a case-control cohort design, serum levels of IL-4 and IL-21 were determined by ELISA in three groups: patients with newly diagnosed PV, patients with chronic, inactive PV (PV in remission), and healthy controls.

Results: The study included 88 individuals (58 women and 30 men), including 26 with newly diagnosed PV, 33 with PV in remission, and 29 healthy controls. A significant difference was found among the groups for IL-21 (P = .044), but not for IL-4 (P = .374). Serum levels of IL-4 and IL-21 in newly diagnosed patients were significantly lower than in healthy controls (P = .005 for both), but these cytokine levels in patients with PV in remission were not different from those of controls (P = .343 and P = .221, respectively). Also, no differences in cytokine levels were detected between the newly diagnosed patients and patients with PV in remission. Regardless of disease phase, we detected significantly lower levels of IL-21 in patients than controls (P = .027), but no differences for IL-4 (P = .374).

Conclusions: IL-4 and IL-21 are involved in PV pathogenesis and disease severity. More studies are required to clarify the role of IL-4 and IL-21 in immunopathogenesis and immune response during PV.
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http://dx.doi.org/10.5826/dpc.1102a35DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8060009PMC
March 2021

Family impact of pemphigus disease in an Iranian population using the Family Dermatology Life Quality Index.

Int J Womens Dermatol 2020 Dec 12;6(5):409-413. Epub 2020 Sep 12.

Autoimmune Bullous Diseases Research Center, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran.

Background: Pemphigus vulgaris (PV) is a rare but seriously disabling disorder of the skin and mucous membranes that can gravely impact the quality of life (QoL) of patients.

Objective: The aim of the present study was to identify how family members of patients with PV are affected by the disease.

Methods: A total of 118 patients with confirmed PV and one of their family members (caregivers) were enrolled in the study. To calculate disease severity, the Autoimmune Bullous Skin Disorder Intensity Score was used. The Persian version of the Dermatology Life Quality Index (DLQI) questionnaire was used to evaluate the QoL of patients and the Family Dermatology Life Quality Index (FDLQI) to evaluate the QoL of caregivers.

Results: The mean age of patients was 43.14 ± 12.5 years. Ninety patients (76.3%) were female. Eighty-one patients (68.6%) had the mucocutaneous phenotype and 37 cases (31.4%) the mucosal phenotype. The DLQI score was 10.1 ± 7.1 for patients. The DLQI score was higher for patients with the mucocutaneous phenotype (11.8 ± 7.5) than those with the mucosal phenotype (6.4 ± 4.9;  < .001). QoL was significantly affected by disease severity. FDLQI score was 13 ± 7 for caregivers, and was significantly higher in older caregivers and married ones. There was a positive correlation between patients' admission frequencies and FDLQI score. FDLQI score was also significantly affected by the Autoimmune Bullous Skin Disorder Intensity Score of patients' disease severity. The QoL of patients and their caregivers showed a significant positive correlation.

Conclusion: The QoL of patients and their families are impaired significantly, and is considerably prominent in the mucocutaneous phenotype of PV and more severe forms.

Limitation: Pemphigus Disease Area Index (PDAI) and Autoimmune Bullous Disease QoL (ABQoL) were not used in this study.
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http://dx.doi.org/10.1016/j.ijwd.2020.09.004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8060665PMC
December 2020

Patchy Alopecia in a Patient with Rheumatoid Arthritis: A Practical Application of Trichoscopy.

Case Rep Dermatol 2021 Jan-Apr;13(1):42-46. Epub 2021 Jan 25.

Autoimmune Bullous Diseases Research Center, Tehran University of Medical Sciences, Razi Hospital, Tehran, Iran.

Trichoscopy is an efficient, convenient, and accurate diagnostic dermatological procedure which is widely used in the examination of patients with skin diseases. Herein, we report a 56-year-old woman with a long-term history of rheumatoid arthritis complaining of pruritic patchy alopecia on her scalp who was referred for biopsy to exclude cutaneous lupus erythematosus. Taking advantage of trichoscopy, we were able to quickly diagnose tinea capitis. Following administration of the proper treatment the disease resolved completely.
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http://dx.doi.org/10.1159/000511536DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7879245PMC
January 2021

Lichen planus-like lesions in 36 patients with pemphigus: A cross-sectional study.

Oral Dis 2021 May 15;27(4):947-951. Epub 2020 Sep 15.

Autoimmune Bullous Diseases Research Center, Tehran University of Medical Sciences, Tehran, Iran.

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http://dx.doi.org/10.1111/odi.13614DOI Listing
May 2021

Drug-induced pemphigus: A systematic review of 170 patients.

Int Immunopharmacol 2021 Mar 5;92:107299. Epub 2021 Jan 5.

Autoimmune Bullous Diseases Research Center, Tehran University of Medical Sciences, Tehran, 009821 Iran.

Pemphigus encompasses a rare heterogeneous group of autoimmune blistering diseases characterized by cutaneous and/or mucosal blistering. Multiple factors, such as some specific types of drugs, have been found to be involved in the induction of pemphigus. Here, we have designed a systematic review by searching PubMed/Medline and Embase databases to find the drugs, involved in pemphigus induction and exacerbation (updated on 19 August 2019). From 1856 initially found articles, 134 studies (198 patients; 170 patients in the drug-induced patients and 28 in exacerbation group) have been included. Regarding drug-induced cases, the mean age was 57.19 ± 16.9-year-old (ranged 8-105), and patients had developed pemphigus within a mean of 154.27 days. Pemphigus vulgaris (38.9%), pemphigus foliaceus (33.5%), and paraneoplastic pemphigus (3.6%) were the most common subtypes. Furthermore, penicillamine (33.1%), captopril (7.7%), and bucillamine (6.5%) were the most reported drugs related to pemphigus induction; penicillamine was associated with the most persistent disease. Regardless of disease subtype, cutaneous, mucocutaneous, and mucosal involvements were reported in 68.6%, 30.1%, and 1.3% of patients, respectively. In total, the IgG deposition in the pathological studies, being positive for autoreactive antibodies in the serum against desmoglein 3 (Dsg3), and desmoglein 1 (Dsg1), were reported in 93%, 34.9%, and 72.7% of reported patients, respectively. Regarding the management of such patients, in 75% of healed cases, treatment (mainly transient systemic and topical corticosteroids and/or azathioprine) was needed besides stopping the probable pemphigus-inducing culprit drug, while drug cessation was enough to control the disease in 25%. As the outcomes, the lesions in 129 of 147 (87.8%) patients had been healed, while in 18 (12.2%), no healing was reported; fifteen out of 18 had died. In conclusion, some specific groups of treatments can induce pemphigus, including penicillamine, captopril, and bucillamine; despite the similar clinical and pathological manifestations to classical pemphigus, most of the cases are less severe and have a better prognosis.
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http://dx.doi.org/10.1016/j.intimp.2020.107299DOI Listing
March 2021

Characteristics and outcomes of COVID-19 in patients with autoimmune bullous diseases: A retrospective cohort study.

J Am Acad Dermatol 2021 04 24;84(4):1098-1100. Epub 2020 Dec 24.

Autoimmune Bullous Diseases Research Center, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran. Electronic address:

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http://dx.doi.org/10.1016/j.jaad.2020.12.043DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7836213PMC
April 2021

Rituximab in practice: Clinical evaluation of patients with pemphigus after rituximab administration.

Dermatol Ther 2021 01 15;34(1):e14633. Epub 2020 Dec 15.

Autoimmune Bullous Diseases Research Center, Tehran University of Medical Sciences, Tehran, Iran.

Pemphigus Vulgaris (PV) is a rare autoimmune blistering disease, which mainly causes mucosal and/or cutaneous lesions. In June 2018, FDA approved Rituximab (RTX)-a B-cell depleting agent-for the management of patients with moderate-to-severe pemphigus. Although the majority of patients respond well to this drug, some do not reach complete remission with a single cycle of RTX. In this review, following an overview of RTX and its clinical outcomes, we have focused on the possible outcomes after RTX therapy in patients with PV. The response is defined into four main categories; complete responders, partial responders, nonresponders, and paradoxical reactions, based on three possibilities of reaching the consolidation phase after 3 months, reaching remission until 6 months, and the ability of corticosteroid tapering in 6 months after RTX administration. Concerning the safety of RTX, three categories of infusion reactions, short and long-term side effects are discussed. Additionally, we have suggested approaches for the evaluation of clinical and serological responses at different critical time-points, including 1, 2, 3, and 6 months after RTX administration. Finally, available markers to predict the response to RTX and research gaps in the field of RTX therapy have been summarized.
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http://dx.doi.org/10.1111/dth.14633DOI Listing
January 2021

Übergang zwischen Pemphigus vulgaris und Pemphigus foliaceus: eine 10-jährige Follow-up-Studie.

J Dtsch Dermatol Ges 2020 Nov;18(11):1302-1305

Department of Dermatology, Autoimmune Bullous Diseases Research Center, Razi Hospital, Tehran University of Medical Sciences (TUMS), Tehran, Iran.

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http://dx.doi.org/10.1111/ddg.14226_gDOI Listing
November 2020

Evaluating the efficacy and safety of topical sirolimus 0.2% cream as adjuvant therapy with pulsed dye laser for the treatment of port wine stain: A randomized, double-blind, placebo-controlled trial.

J Cosmet Dermatol 2020 Nov 29. Epub 2020 Nov 29.

Autoimmune Bullous Diseases Research Center, Tehran University of Medical Sciences, Tehran, Iran.

Background: Port Wine Stain (PWS) is a congenital capillary malformation. Although multiple treatments are required, the gold standard treatment for PWS is Pulsed Dye Laser (PDL). Given its anti-angiogenic effects, sirolimus can be considered as an adjuvant to PDL in PWS.

Aim: To evaluate the efficacy and safety of topical sirolimus (Rapamycin) 0.2% cream as adjuvant therapy for PDL for PWS.

Methods: In this randomized double-blind placebo-controlled trial, 15 patients with PWS were enrolled. Each lesion was divided into upper and lower parts, and each part was assigned randomly to receive PDL (4 sessions, 2 months apart) plus sirolimus vs PDL and placebo. The response was evaluated using colorimetry, investigator global assessment (IGA), and patient global assessment (PGA) every two months for eight continuous months.

Results: According to the colorimetric analysis, medial and lateral sides of the treatment and placebo parts did not differ significantly (both P-value > .05). However, according to PGA and IGA, there was a significant difference in favor of sirolimus (P-values = .041 and .039, respectively). Itching and dryness (86.7%), contact dermatitis (20%) were the most common adverse effects in the sirolimus group, while none of them were observed in placebo.

Conclusion: Although the improvement was significant subjectively, topical sirolimus 0.2% as an adjuvant to PDL does not appear to improve PWS erythema using calorimetric assessment.
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http://dx.doi.org/10.1111/jocd.13867DOI Listing
November 2020

Clinical and dermatoscopic characteristics of lichen planus-like keratosis in a West-Asian population.

Australas J Dermatol 2021 Feb 17;62(1):e55-e61. Epub 2020 Nov 17.

Primary Care Clinical Unit, Faculty of Medicine, The University of Queensland, Herston, Australia.

Background/objective: The clinical and dermatoscopic features of lichen planus-like keratosis have been described but the characteristics of this entity in a West-Asian population are not known.

Methods: We retrospectively analysed 82 histopathologically verified cases of lichen planus-like keratosis from 81 patients from Iran and Turkey.

Results: The majority of lichen planus-like keratoses were macules (61% n = 50), clinically pigmented (67.1% n = 55) and dermatoscopically multi-coloured (91.5% n = 75). The majority (63.4%) had a single dermatoscopic pattern, most frequently: structureless (35.4%), dots (14.6%) and angulated lines (8.5%). Of the lesions with more than one pattern (n = 30), the majority (n = 21) had asymmetry of pattern, the most common combinations being structureless plus dots (n = 8) and structureless plus angulated lines (n = 5). The most common structure was pigmented dots, most frequently grey and present in 70.7% of cases. Vessels were seen in 30.5% of lesions, being significantly more prevalent in non-pigmented, than pigmented, lichen planus-like keratoses (83.3% vs. 21.4% P < 0.001). When we compared lichen planus-like keratosis in the current study to that entity in a large North American study, the statistically significant differences in a West-Asian population included a greater frequency of pigmented variants, a lower incidence in females and a lower prevalence on the torso, in favour of the face.

Conclusions: Lichen planus-like keratosis in a West-Asian population has clinical and dermatoscopic similarities to that entity in another studied population. The significant differences in gender association and anatomical site may be secondary to cultural factors.
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http://dx.doi.org/10.1111/ajd.13455DOI Listing
February 2021

Immunosuppressive drugs for patients with psoriasis during the COVID-19 pandemic era. A review.

Dermatol Ther 2021 Jan 10;34(1):e14498. Epub 2020 Nov 10.

Department of Dermatology, Razi Dermatology Hospital, Tehran University of Medical Sciences, Tehran, Iran.

The COVID-19 has been spreading around the world. Concerns about the safety of administration of immunosuppressive drugs have been raised for treatment of psoriasis (PSO), and there is insufficient evidence for the risk of COVID-19 infection for psoriatic patients using these drugs, so we did a review, focusing on the risk of overall infection associated with the most commonly used immunosuppressive drugs, such as methotrexate, biologics, cyclosporin, Janus kinase inhibitors for the treatment of PSO. The data on the effect of immunosuppressive drugs on this virus may be ever-changing and remains to be clear. We recommend the initiation and continuation of low-risk immunomodulating drugs, such as Interleukin (IL)-17, IL-12/23, and IL-23 inhibitors, for treatment of PSO during COVID-19 era. For psoriatic patients with comorbidities switching to safer modalities such as systemic retinoids, apremilast, and home phototherapy is recommended. Immunosuppressive drugs should be withheld in psoriatic patients with the COVID-19 infection.
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http://dx.doi.org/10.1111/dth.14498DOI Listing
January 2021

Estimated cut-off values for pemphigus severity classification according to pemphigus disease area index (PDAI), autoimmune bullous skin disorder intensity score (ABSIS), and anti-desmoglein 1 autoantibodies.

BMC Dermatol 2020 10 31;20(1):13. Epub 2020 Oct 31.

Autoimmune Bullous Diseases Research Center, Tehran University of Medical Sciences, Razi Hospital, Vahdate-Eslami Square, Tehran, 11996, Iran.

Background: Pemphigus is a potentially fatal disease if left untreated. Valid scoring systems and defined cut-off values for classification of patients would help with better management through specified pharmaceutical and non-pharmaceutical treatments.

Methods: In this study, pemphigus patients who were receiving immunosuppressive treatments and had recent disease relapse were recruited for examination of pemphigus disease area index(PDAI), autoimmune bullous skin disorder intensity score (ABSIS), physician global assessment (PGA), autoimmune bullous disease quality of life (ABQoL), anti-desmoglein 1 (anti-Dsg1), and anti-Dsg3 autoantibody titers from December-2017 to February-2018. Cut-off values were estimated using model-based clustering classification and the 25th and 75th percentiles approach, performed separately for the exclusive cutaneous, exclusive mucosal, and mucocutaneous groups.

Results: In the 109 included patients, the 25th and 75th percentiles cut-offs were 6.2 and 27 for PDAI score, and 4 and 29.5 for ABSIS score. The model-based analysis resulted in two groups (cut-point:15) for PDAI score, and three groups (cut-points:6.4 and 31.5) for ABSIS score. The groups were significantly different for the PDAI, ABSIS, PGA, and ABQoL values. Based on anti-Dsg1 autoantibody values, the model-based analysis cut-point was 128 and the 25th and 75th percentiles cut-offs were 98 and 182. Anti-Dsg3 autoantibody values did not differentiate between pemphigus severity classes.

Conclusions: Estimated cut-off values based on the anti-Dsg1 level, PDAI, and ABSIS scoring systems could be used to classify patients into different severity grades for better management and prognosis.
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http://dx.doi.org/10.1186/s12895-020-00105-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7603731PMC
October 2020

Concomitant bullous pemphigoid and palmoplantar keratoderma: A report of three cases and review of literature.

Dermatol Ther 2020 11 9;33(6):e14481. Epub 2020 Nov 9.

Autoimmune Bullous Diseases Research Center, Tehran University of Medical Sciences, Tehran, Iran.

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http://dx.doi.org/10.1111/dth.14481DOI Listing
November 2020

Acute generalized exanthematous pustulosis with a focus on hydroxychloroquine: A 10-year experience in a skin hospital.

Int Immunopharmacol 2020 Dec 19;89(Pt B):107093. Epub 2020 Oct 19.

Autoimmune Bullous Diseases Research Center, Tehran University of Medical Sciences, Tehran 63911-11996, Iran. Electronic address:

Objective: Acute generalized exanthematous pustulosis (AGEP) is a severe skin pustular drug reaction that can lead to life-threatening consequences. In this study, we have investigated the characteristics and outcomes of patients with AGEP in a tertiary skin hospital.

Methods: From March 2007 to December 2019, medical records of all patients diagnosed with AGEP, were assessed. Demographic data, culprit drug, past medical history, laboratory tests, recurrence, and systemic organ involvement were all documented as well.

Results: Seventy-four patients, including 54 women (73%) and 20 men (27%), with a mean age of 44.3 ± 16.5 years were evaluated. The most common comorbidities among the patients were rheumatoid arthritis and diabetes. In addition, hydroxychloroquine, cephalosporin, and amoxicillin were found as the three most common medications associated with AGEP induction. Among the study group, seventeen (23%) patients had systemic organ involvement (nine (12.2%), six (8.1%), and five (6.8%) had hepatic, renal and pulmonary involvement, respectively). All patients responded to oral prednisolone within a median of five days (IQR = 4; ranged 2-14). The median duration of treatment was significantly longer in hydroxychloroquine group compared to other drugs (8 versus 5 days; HR 0.57,95%CI 0·35-0.91). Likewise, the median duration of treatment was significantly longer in febrile patients compared to the afebrile ones (7 versus 4 days; HR 0.46, 95%CI 0.25-0.85). Recurrence occurred in six patients after resuming treatment with the same medication. The mean Naranjo score was 7.6 ± 0.9 denoting a probable causal relationship.

Conclusion: In this study, we found that using hydroxychloroquine and presence of fever are the risk factors potentially leading to a prolonged treatment duration of AGEP.
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http://dx.doi.org/10.1016/j.intimp.2020.107093DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7572086PMC
December 2020

Investigating expression pattern of eight immune-related genes in pemphigus patients compared with the healthy controls and after rituximab therapy: Potential roles of CTLA4 and FCGR3A genes expression in outcomes of rituximab therapy.

Dermatol Ther 2020 11 27;33(6):e14380. Epub 2020 Oct 27.

Department of Medical Genetics, Faculty of Medicine, Tehran University of Medical Sciences, Tehran, Iran.

Pemphigus is a rare group of autoimmune diseases, which its exact molecular pathogenesis and therapeutic biomarkers remained unknown. In this regard the expressions of eight immune-related genes was evalualted in pemphigus patients. Forty-six pemphigus patients, either new case or on minimal therapy, were recruited. The expressions of IL22, IL9, IL21, EBI3, TNFSF13B, FCGR3A, CTLA4, and PDCD1 genes were analyzed at baseline, compared with 32 healthy controls, and their changes were monitored 3 months after rituximab (RTX) therapy through Reverse Transcriptase Real-time PCR (RT-Real-time PCR). Except of IL21, which was similar in both groups, expressions of other genes were significantly lower in patients compared with the controls (P-value <.05). PDCD1, EBI3, IL21, and IL22 genes were significantly overexpressed three months following RTX administration (P-value <.05). Higher prednisolone dosage and PDAI-score were positively correlated with CTLA4 and FCGR3A expressions after 3 months, respectively (P-value = .019 and .048, respectively). Anti-desmoglein 1 (Dsg 1) titer and its positivity at baseline were associated with TNFSF13B expression, FCGR3A expressions, and the PDAI-score. Our results suggest the possible involvement of some gene expressions in pemphigus immunopathogenesis, which could be affected by RTX therapy and also might be used as prognostic biomarkers.
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http://dx.doi.org/10.1111/dth.14380DOI Listing
November 2020

Intralesional injection of biosimilar rituximab in recalcitrant mucocutaneous lesions of patients with pemphigus vulgaris: A pilot study.

Dermatol Ther 2020 11 30;33(6):e14407. Epub 2020 Oct 30.

Department of Dermatology, Razi Hospital, Tehran University of Medical Sciences (TUMS), Tehran, Iran.

The efficacy of intravenous rituximab (RTX) in patients with pemphigus have been shown in the previous studies. The study aimed to investigate the effectiveness of intralesional injection of RTX in the healing of pemphigus lesions, with lower doses and probable better safety profile than intravenous RTX. Eleven Pemphigus patients with recalcitrant lesions received two intralesional injections of biosimilar RTX, 5 mg/cm . During 6 months follow-up, Pemphigus Disease Area Index, the patients' satisfaction, quality of life, the disease activity, the number and size of lesions, the anti-desmoglein (Dsg) 1 and 3 antibodies and the count of CD4+ and CD19+ cells were assessed. All patients were in partial remission on therapy. The absolute count of CD19+ B cells showed a statistically significant decline (P = .006). The percentage of CD4 + T lymphocytes increased 2 weeks after injection and decreased 2 weeks later (P = .01). The average number and size of lesions decreased. The concentration of anti-Dsg 3 antibody decreased insignificantly during the study. The severe pain during the injection was considered as the main complication. At the end of the study, two patients were in complete remission on therapy, and the other nine remained in partial remission on therapy. Few side effects resulting from intralesional injection of RTX and enhanced quality of life of the patients were considered as the valuable achievements of this study. The results showed that although a low dose of RTX leads to a significant decrease of CD19+ B lymphocytes, it did not show parallel clinical effectiveness.
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http://dx.doi.org/10.1111/dth.14407DOI Listing
November 2020

Rituximab monotherapy in mild pemphigus.

J Dermatolog Treat 2020 Oct 25:1-3. Epub 2020 Oct 25.

Autoimmune Bullous Diseases Research Center, Tehran University of Medical Sciences, Tehran, Iran.

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http://dx.doi.org/10.1080/09546634.2020.1836309DOI Listing
October 2020

Pemphigus scalp lesions: is trichoscopy helpful in clinical diagnosis?

Int J Dermatol 2021 Jan 5;60(1):81-87. Epub 2020 Oct 5.

Autoimmune Bullous Diseases Research Center, Department of Dermatology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran.

Background: Pemphigus is an autoimmune intraepidermal bullous disease which can affect skin and∕or mucous membranes. Trichoscopy is a non-invasive tool that can help in the clinical diagnosis especially if the patient is presented with only scalp lesions. We conducted this study to investigate the trichoscopic features of pemphigus scalp lesions.

Methods: A total of 100 patients with confirmed diagnosis of pemphigus entered the study; Seventy-nine patients were diagnosed as pemphigus vulgaris (PV) and 21 as pemphigus foliaceus (PF). Trichoscopy of the scalp lesions was performed, and three dermatologists examined separately the trichoscopic images of each patient. The results were re-checked simultaneously by the examiners. Association between variables was analyzed by using different tests including: chi-square test or Fischer's exact test, independent T-Test or Mann-Whitney nonparametric test and logistic regression model when applicable.

Results: Mean age of the patients was 45.41 ± 11.13 years, and 58% of cases were male. The most common trichoscopic features observed among all cases were diffuse scaling (71/100), presence of any types of vessels (67/100), and extravasation (63/100). Positive anagen pull test, extravasation, yellow hemorrhagic crust, and polymorphic vessels were more commonly seen in PV than PF cases (P value < 0.05), and by having data of these variables, we can differentiate between PV and PF scalp lesions with 74% sensitivity and 91% specificity.

Conclusion: Trichoscopy can help in clinical diagnosis of pemphigus scalp lesions but should be followed by other modalities such as biopsy and direct immunofluorescence for definite diagnosis.
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http://dx.doi.org/10.1111/ijd.15231DOI Listing
January 2021

Autoimmune Bullous Disease Quality of Life (ABQoL) questionnaire: Validation of the translated Persian version in pemphigus vulgaris.

Int J Womens Dermatol 2020 Sep 26;6(4):306-310. Epub 2020 Mar 26.

Autoimmune Bullous Diseases Research Center, Tehran University of Medical Sciences, Tehran, Iran.

Background: Autoimmune bullous diseases are a group of rare, chronic, blistering diseases, with pemphigus vulgaris (PV) being the most common type in Iran. Skin and mucosal involvement and therapy may have a dismal impact on the quality of life of affected patients.

Objective: We aimed to assess the validity and reliability of the Farsi (Persian) version of the Autoimmune Bullous Quality of Life (ABQoL) index in Iranian patients with PV.

Methods: Forward and backward translation procedures were used to prepare the Persian version of the ABQoL questionnaire. A total of 180 patients were asked to complete the questionnaires, and 24 cases repeated the test after 2 weeks. For validity and reliability, an exploratory factor analysis was performed along with a parallel analysis to determine the number of factors. The multi-trait, multi-method matrix assessed convergent and discriminant validity. To evaluate internal reliability and reliability over time, Cronbach's alpha and intraclass correlation coefficients were reported.

Results: Two factors explained a total variance of 54.9% in the data. The first and second factors comprised questions 1-3, 5, 7, 9, and 12-17 (symptoms-social) and questions 4, 6, 8, 10, and 11 (mucosal-psychological), respectively. The internal reliability of the Persian version of the ABQoL index was also acceptable, with a Cronbach's alpha of .855 for total items, .918 for the first factor, and .6 for the second factor. Our results suggest an acceptable convergent and discriminant validity of the Persian version of the ABQoL questionnaire.

Conclusion: The Persian version of ABQoL index is a valid and reliable tool to monitor quality of life in patients with PV. Further studies are needed to assess the sensitivity of this instrument to changes in different disease activity and correlation with more general tools for the measurement of quality of life.
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http://dx.doi.org/10.1016/j.ijwd.2020.03.043DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7522916PMC
September 2020

Adverse electrocardiographic effects of rituximab infusion in pemphigus patients.

Dermatol Ther 2020 11 20;33(6):e14299. Epub 2020 Sep 20.

Department of Cardiology, Imam Khomeini Hospital Complex, Tehran University of Medical Sciences, Tehran, Iran.

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.
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http://dx.doi.org/10.1111/dth.14299DOI Listing
November 2020

Authors' reply to the comment "Treatment considerations for patients with pemphigus during the COVID-19 pandemic".

J Am Acad Dermatol 2021 01 4;84(1):e61-e62. Epub 2020 Sep 4.

Department of Dermatology, Mayo Clinic, Rochester, Minnesota. Electronic address:

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http://dx.doi.org/10.1016/j.jaad.2020.09.002DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7473138PMC
January 2021

Comparing efficacy and safety of potassium hydroxide 5% solution with 5-fluorouracil cream in patients with actinic keratoses: a randomized controlled trial.

J Dermatolog Treat 2020 Sep 24:1-7. Epub 2020 Sep 24.

Autoimmune Bullous Diseases Research Center, Tehran University of Medical Sciences, Tehran, Iran.

Background: Actinic keratosis (AK) is a pre-cancerous skin lesion, associated with development of squamous cell carcinoma. Current treatment options are limited.

Objectives: To compare the efficacy and safety of topical 5-fluorouracil cream (5-FU) and potassium hydroxide 5% (KOH) in the treatment of AK.

Methods: Eighteen patients with AK applied KOH solution or 5-FU on each side of their scalp/face, randomly. The efficacy and safety of these treatments were compared.

Results: Thirteen (118 lesions) and ten (83 lesions) patients were successfully followed for one and three months, respectively. After one month, KOH showed a better clinical response (81% vs. 58%; -value = 0.007) and dermoscopic response (KOH, 65% vs. 5-FU, 46%; -value = 0.04); while no differences were noted after three months (clinical response, 83% vs.70%, -value = 0.1; dermoscopic response, 76% vs. 59%, -value = 0.1). No significant differences in the recurrence rate of the lesion between the two groups were noted at the end of the third month (-value = 0.5). Regarding the safety of the treatments, the risk of developing erythema, scaling, sand swelling was higher in 5-FU group (-value < 0.0001, for all), while more patients in KOH group had erosion and ulcer (-value < 0.001 for both). KOH was up to 96% less expensive than 5-FU.

Limitations: Low number of patients and short-term follow-up limited the analysis.

Conclusion: KOH solution offers a faster and less expensive resolution of AK lesions than does 5-FU.

Clinical Trial Code (irct.ir): IRCT20180909040978N1.
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http://dx.doi.org/10.1080/09546634.2020.1817839DOI Listing
September 2020

Transition between pemphigus vulgaris and pemphigus foliaceus: a 10-year follow-up study.

J Dtsch Dermatol Ges 2020 Nov 24;18(11):1302-1304. Epub 2020 Aug 24.

Department of Dermatology, Autoimmune Bullous Diseases Research Center, Razi Hospital, Tehran University of Medical Sciences (TUMS), Tehran, Iran.

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http://dx.doi.org/10.1111/ddg.14226DOI Listing
November 2020