Factors associated with the healing time of pemphigus vulgaris oral lesions: A prospective study.

OBJECTIVES: This study determines the healing time of lesions on different locations and the contributing factors to the healing time in patients with pemphigus. METHODS: In this prospective study, newly diagnosed patients with mucosal lesions were included. A dermatologist evaluated the lesions, disease status, side effects and assigned the PDAI. Follow-up visits were conducted monthly until the patient reached complete remission and every three months thereafter. A Tzanck smear was performed on lesions clinically suspected to be herpetic in origin. RESULTS: Sixty patients enrolled in the study with a mean age of 45.9 ± 11.7 years. The buccal lesions took the longest to resolve (73[33.5-105.5] days). However, the posterior pharynx lesions showed the shortest healing time (20[13.0-25.5] days). The likelihood of improvement in buccal and soft palate lesions decreased by 5% and 3% with each additional year of age, respectively. Also, the resolution duration of soft palate lesions was significantly shorter in female patients than males (median of 24.0 days vs. 38.5 days). In contrast, lower gingival lesions resolve significantly faster in male patients by a median of 9 days. Herpes simplex virus infection increases the healing time of lesions by 26 days (median of 55 days vs. 29 days, hazard ratio 2.62, 95% CI: 1.04-5.92). CONCLUSIONS: Buccal and lower gingival lesions are more recalcitrant to treatment, while posterior pharynx lesions heal most rapidly. Furthermore, older age was also associated with a lower rate of lesion improvement.

ODSS vs. ABSIS and PDAI oral parts in pemphigus vulgaris: inter-rater reliability and testing times.

OBJECTIVES: The existence of standard methods for diagnosis and measuring the severity of diseases leads to a more accurate severity assessment, the possibility of following up, and the possibility of comparing the results of studies. This study aimed to compare different pemphigus vulgaris (PV) assessment methods regarding inter-observer reliability and testing times-focusing on oral parts. MATERIALS AND METHODS: Two dermatologists evaluated orally involved PV patients by oral parts of Autoimmune Bullous Skin Disorder Intensity Score (ABSIS), Pemphigus Disease Area Index (PDAI), and Oral Disease Severity Score (ODSS). RESULTS: Seventy patients completed the study. The intraclass correlation coefficient showed the evaluators' agreements on ABSIS, PDAI, and ODSS with 0.98, 0.94, and 0.95, respectively. Reliability analyses showed near-perfect relationships between each scoring methods pairs. There was no association between lesion sites and disease severity. The PDAI scoring duration was significantly shorter, and the ABSIS scoring duration was significantly longer. CONCLUSION: ODSS is valid for evaluating oral involvement in patients with PV and relates to ABSIS and PDAI almost perfectly. Besides, it was shown that the evaluation of patients' oral involvement based on PDAI and ODSS is done in about 1 min, which seems clinically reasonable.

The role of SARS-CoV-2 infection and its vaccines in various types of hair loss.

The prevalence of hair loss has increased during COVID-19. In this study, we review the current literature on incidence and characteristics of various types of COVID-19-related and COVID-19-vaccine- related hair loss including telogen effluvium, alopecia areata, friction alopecia and anagen effluvium. Regarding most of them, the more severe the infection, the more profound and prolonged the course of alopecia. However, the most important issue is reassuring the patients of the non-serious nature of this complication, since psychological support is the most important factor in the earlier resolution of the condition.

COVID vaccination in patients under treatment with rituximab: A presentation of two cases from Iran and a review of the current knowledge with a specific focus on pemphigus.

SARS-COV2 vaccines were approved without long-term monitoring due to emergent situations. This has raised some issues about the timing and protocol of receiving vaccines in specific situations such as patients receiving immunomodulatory agents including rituximab, which is widely used for various disorders such as multiple sclerosis, pemphigus, and many rheumatologic disorders. We described two cases of pemphigus vulgaris (a new case and one with flare-up) following vaccination with Astrazeneca in Iran and reviewed the existing data in this regard through searching on PubMed, Google Scholar, and Scopus. All of the relevant papers published until June 28, 2021, which we could access their full-texts were included. We found some recommendations made by rheumatologists, neurologists, and dermatologists in regard to vaccination timing in this group of patients and tried to summarize them to provide a practical guide for clinicians. Clinicians should perform a careful, individualized risk-benefit assessment for their patients and consider a delay in rituximab administration after completion of COVID vaccination if there is not any considerable risk of disease relapse or organ failure. Moreover, choosing vaccines with potential of providing protection after single dose, especially in countries with limited access to vaccines may be a reasonable approach.

SARS-CoV-2 vaccination and practical points in psoriasis patients: A narrative review.

SARS-CoV2 vaccines were approved without long-term monitoring due to emergent situations. This has raised some issues about timing and protocol of receiving vaccines in specific situations including patients with chronic inflammatory disorders such as psoriasis. Here, we present different aspects of SARS-CoV-2 infection and vaccination in psoriasis patients and aim to provide solutions to overcome the potential challenges. In brief, the benefits of vaccination outweigh the potential risk; vaccine-triggered de novo or flares of psoriasis is uncommon. As such, all psoriasis patients, especially those receiving systemic treatments including anti tumor necrosis factor agents, are strongly recommended to get SARS-CoV-2 vaccines. It is recommended that new immunosuppressive/immunomodulatory therapies be initiated at least 1 week after the second SARS-CoV-2 vaccine dose, if possible. In addition, in severe and active forms of psoriasis, it is better to delay vaccination until stabilization of the disease.

Botulinum toxin injection as a single or combined treatment with non-cross-linked high molecular weight and low molecular weight hyaluronic acid gel for neck rejuvenation: A randomized clinical trial.

The unpleasant appearance of the neck through the aging process could burden the psychosocial quality of life, therefore various treatment modalities have been used to rejuvenate the neck. The present study aimed to evaluate the efficacy of botulinum toxin injection as a single or combined treatment with Profhilo gel for neck rejuvenation. Patients with a request for neck rejuvenation meeting the inclusion criteria were randomly divided into two groups. Initial clinical scoring based on Wrinkle Severity Rating Scale (WSRS) and skin biomechanical parameters using a multiadaptor system (MPA) were performed before starting the treatment. Dysport Botox was injected for all of the participants, moreover, in one of the groups (intervention), Profhilo gel was injected after 2 weeks and a follow-up visit was performed for both groups 3 months later. Patients were evaluated according to the GAIS (global aesthetic improvement scale) by two blind dermatologists and skin biophysical parameters were measured by MPA such as TEWL, hydration, thickness, density, visco-elasticity, net elasticity, pliability/firmness. In this study, 19 participants in the control (Dysport) and intervention (Dysport + PROFhilo gel) groups were evaluated. Before starting the treatment, the two groups were similar in terms of WSRS scores and biophysical skin parameters. Three months after the treatment, it was observed that Hydration (p < 0.001) and Thickness of the skin (p = 0.028) in the intervention group were significantly higher than in the control group. Similarly, the GAIS scores of the intervention group showed significantly better results comparing to the control group (p < 0.001). Profhilo gel following Dysport Botox can be considered as a safe and more effective treatment of neck aging comparing to Dysport Botox injection alone in patients who are not a candidate for surgery due to any reason.

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.

Multiple cycles of rituximab therapy for pemphigus: A group of patients with difficult- to-treat disease or a consequence of late rituximab initiation?

Pemphigus is a serious autoimmune disease with few appropriate therapeutic options. Although rituximab (RTX) has recently shown great promise in this regard, the best protocol of its administration is remains to be elucidated. This study aimed to evaluate the patients who need at least 3 cycles of treatment with RTX to identify hard-to-treat patients' characteristics, which might lead to consider more prompt protocols for treatment of them. A retrospective cross-sectional study was conducted on 45 patients with pemphigus vulgaris who received at least 3 cycles of RTX. Their demographic, clinical, and laboratory data as well as details of treatment protocol and final clinical situation of patients were evaluated. Totally, 45 patients (21 men and 24 women) with mean age of 44.33 years were included in this paper. Women were about 8 years older than men (mean age: 48.1 years versus 40.1 years, p: 0.011) and needed RTX approximately 2 years later in their course of disease (gap: 41.04 months vs. 14.85 months, p: 0.003). Buccal, truncal, and scalp regions were the most frequent sites of involvement respectively. A significant decrease in both anti-Dsg1, 3 was seen at last visit compared to baseline. However, the amount of this decrement was not significantly different between them (p: 0.083). Partial remission in 31.11%, complete remission in 24.44%, relapse in 15.56%, partial remission on treatment in 15.56% and complete remission on treatment in 13.33% were seen at the last follow-up session. RTX is an effective medication for PV even in patients with refractory disease and its therapeutic effect is increased with each subsequent cycle. Male gender, severe oral mucosal involvement on disease onset and extensive scalp and truncal lesions as first cutaneous manifestation of disease are more likely to be signs of refractory PV. Hence, it is reasonable to consider more prompt protocols for treatment of these cases. Moreover, late prescription of RTX during the course of disease might play a role in presence of more resistant form of disease.

Retrospective study of gingival involvement in pemphigus: A difficult to treat phenomenon.

Pemphigus is a group of autoimmune diseases characterized by flaccid lesions on the skin and mucous membranes. In pemphigus vulgaris, the most common subtype of pemphigus, lesions might be appeared anywhere on the oral mucosa, mostly in the buccal mucosa. However, the gingiva is a less frequently affected site. Here, we performed a retrospective study at Tehran University of Medical Sciences, covering a two-year period to identify pemphigus patients with active lesions confined to the gingiva. Considering 1787 initially evaluated pemphigus cases, 512 (28.6%) were found to have a history of gingival involvement. Among them, 31 patients had only gingival involvement during their last visit, including 29 (93.5%) women and only two (6.5%) men. The mean of disease duration in this group was 5.29 ± 3.46 years, and they had gingival involvement for a mean of 23.9 ± 19.3 months. Of 28 patients, nine were negative for anti-Dsg3 and 24 were negative for anti-Dsg1. In 24 patients, who received rituximab, the mean pemphigus disease area index specifically for gingiva was 4.76 ± 0.74 at baseline, which had changed to 4.13 ± 0.75 and 3.26 ± 0.63 three and 6 months after rituximab administration, respectively. After 3 months, gingival lesions were either entirely resolved (n = 3), partially resolved (n = 11), remained unchanged (n = 2), or progressed (n = 7). Gingiva-confined presentation of lesions in pemphigus could be non-anti-Dsg1/3 dependent in some patients. Such patients do not respond well to conventional treatments and rituximab therapy. More studies on the pathogenesis of gingiva-confined presentation of pemphigus are required.

Evaluating the risk-to-benefit ratio of using cotrimoxazole as a pneumocystis pneumonia preventative intervention among pemphigus patients treated with rituximab: A retrospective study with 494 patients.

Rituximab is widely used as the first-line treatment for pemphigus patients. Since it depletes the B cells, it increases the risk of infections. Here, we evaluated the prophylactic efficacy of cotrimoxazole in decreasing the risk of pneumocystis pneumonia (PCP) infection in the pemphigus patients treated with rituximab. The medical records of confirmed pemphigus patients receiving rituximab were evaluated in two groups; those who received cotrimoxazole as a prophylactic after rituximab and patients who only received rituximab without any prophylaxis. The occurrence of PCP infection was determined in each group and compared. Medical records of 494 patients, including 301 women and 193 men, with the mean age of 46.74 years were analyzed. The phenotypes of the disease were mucocutaneous (n = 364), mucosal (n = 88), and cutaneous (n = 42). Among them, 235 cases had received cotrimoxazole as a prophylaxis and 259 patients did not. The incidence of PCP in total patients was 2 (0.4%), one in each group. Accordingly, no significant difference was observed in the incidence of PCP between two groups (p = 0.84). Also, no cotrimoxazole-related side effect was observed in the treated group. It seems that due to the low incidence of PCP in pemphigus patients treated with rituximab, prophylactic cotrimoxazole therapy is not necessary and it only increases the overall therapy cost and might cause cotrimoxazole-related adverse effects in some patients. However, regarding its probable beneficial effect in patients with long-term history of immunosuppressive therapy, more studies are required.

Prevalence and management of chronic hepatitis B in pemphigus and pemphigoid patients: New evidence for the safety of rituximab.

Although the treatments of pemphigus and pemphigoid patients have tended toward safer options, patients with chronic infections seem to be still at the risk of infection reactivation when they are exposed to any of immunosuppressive treatments. A retrospective study on 1646 registered pemphigus and pemphigoid patients was conducted between January 2017 and February 2019 and the prevalence of HBV, the association between the treatments, mainly prednisolone and rituximab with HBV reactivation as well as outcomes of patients after management with antiviral therapies were evaluated. From 1646 reviewed patients, 10 (0.60%) patients with chronic HBV were identified. We found a negative correlation between the ALT (p-value<0.001), AST (p-value = 0.090), and Pemphigus Disease Area Index (PDAI) (p-value = 0.034) and age of patients. At the time points that prednisolone dosage was higher, higher levels of ALT, but no difference in AST levels was noted. The portion of patients with normal ALT was significantly higher (p-value = 0.036; OR = 2.22) in those who had received rituximab within the previous 6 months (38 of 49; 77.6%) as compared to those who did not (81 of 133; 60.9%). We concluded that avoidance (high dose) systemic corticosteroids in patients with chronic HBV, and using rituximab instead in severe cases benefit this group of patients.

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

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

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

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

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

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

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

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.

Does Double-Pass Pulsed-Dye Laser With Long and Short Pulse Duration Increase Treatment Efficacy of Port-Wine Stain? A Randomized Clinical Trial.

BACKGROUND: Although pulsed-dye laser (PDL) 595 nm is known as the gold standard for treatment of port-wine stains (PWS), complete clearance of lesions occurs in a minority of cases. OBJECTIVE: To compare the efficacy and safety of double-pass pulsed-dye laser (DPL), long pulse duration (20 m/s) followed by short pulse duration (1.5 m/s) within 20 minutes interval, with single-pass pulsed-dye laser (SPL) for (1.5 m/s) in the treatment of PWS. METHODS: Twenty-four patients with PWS underwent 3 sessions of PDL. Each lesion was randomly divided into 2 portions to receive DPL or SPL. Colorimetric and dermoscopic evaluations were used to determine the response objectively. In addition, improvement was scored subjectively using the visual analog scale (VAS). RESULTS: According to colorimetric analysis, the mean blanching rates for DPL and SPL treated sites were 48% (SD = 0.215) and 37% (SD = 0.213), respectively (p = .001). With VAS, 3.79 (SD = 0.93) and 3.33 (SD = 0.91) improvement scores were reported in the DPL and SPL treated areas, respectively (p = .008). Dermoscopic images showed that larger deep vessels were the most common remnant vessels in both treatment areas. CONCLUSION: Compared with SPL, DPL with 20 minutes interval seems to be a more effective and safe method for the treatment of PWS.

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.

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/m2 or 2 × 500 mg) and modified-dose (3 × 375 mg/m2 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.

Pathogenic and protective roles of cytokines in pemphigus: A systematic review.

Pemphigus is a rare and life-threatening group of blistering autoimmune diseases that affects the skin and mucous membranes. There are two major subtypes of pemphigus, including pemphigus vulgaris (PV) and pemphigus foliaceus (PF); each has different clinical manifestations. Pemphigus cannot be considered as a single disease and each patient may have a specific immunological profile. There are a lot of studies available regarding the role of different cytokines in the pathogenesis of pemphigus, although the data are not coherent between different studies. In this study, a systematic review from inception to December 25, 2019, through the MEDLINE/PubMed database had been performed to address several aspects of cytokines' roles in PV and PF. As a result, 57 studies from 352 initially found records, containing 26 cytokines had met the inclusion criteria. We found different pieces of evidence in favor of increased levels of TNF-α, TGF-ß, interleukin (IL)-8, IL-10, IL-12, IL-17, IL-21; while decreased levels of IL-2 and IL-23 in pemphigus patients. Regarding other cytokines, such as IFN-γ, IL-5, IL-15, IL-22, there are controversial results. Different studies suggested the association of TNF-α and IL-6 with disease activity and autoantibodies values. However, there is uncertainty regarding the role of IFN-γ, IL-2, IL-15, IL-27, and IL-33. Treatments with immunosuppressive agents may decrease IL-1, IL-6, IL-8, IFN-γ, IL-33, IL-17 levels. In conclusion, cytokines are deeply involved in PV and PF pathogenesis, and targeting specific cytokines may lead to development of more promising treatments for pemphigus.

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

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/cm2 . 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.

Diagnostic value of cytology in oral ulcer and comparison with histopathology and direct immunofluorescence.

Rapid diagnosis of pemphigus vulgaris (PV) is an important task in patient's prognosis and treatment. Although PV is routinely diagnosed through investigation of pathology specimens and direct immunofluorescence assays, Tzanck smear can be used as rapid, inexpensive, and easily used test to confirm its clinical diagnosis. This study aimed to determine the diagnostic value of Tzanck smear in erosive oral lesions of PV and also determine its sensitivity and specificity for diagnostic purposes. A total of 68 patients with erosive/ulcerated oral lesions were included in this study and divided into PV (case group) vs other causes of erosive oral lesions (control group). From all participants, two Tzanck smears were prepared for both Giemsa and hematoxylin-eosin (H&E) staining. For definite diagnosis, histopathology and direct immunofluorescence evaluations were performed based on clinical findings. The sensitivity of acantholytic cells in Tzanck smear of erosive oral lesions of PV cases was 80.5% (for both Giemsa and H&E staining), whereas specificity values of Giemsa and H&E staining were 84.6% and 96.3%, respectively. Based on our findings, the Tzanck smear of erosive oral lesions is a simple, quick, and inexpensive test for screening and primary diagnosis of PV.