Treatment Of Pemphigus Vulgaris Research Articles

Penfhigus Vulgaris: Report Of A Clinical Case

The current article analyzes the topic of pemphigus vulgaris, which is described as an autoimmune disease affecting the skin and mucous membranes, characterized mainly by the formation of superficial blisters on the skin and oral mucosa. The objective was to present a case study of a patient with pemphigus vulgaris. A qualitative and descriptive investigation was carried out in this study. The 40-year-old male patient with pemphigus vulgaris had a complex but satisfactory evolution. Following an exacerbation in July 2023 and complications such as oral ulcers and ocular issues, he was hospitalized again. Aggressive treatment was initiated with medication adjustments, resulting in significa[1]nt improvement with remission of skin lesions, pain control, and no signs of infection. After multidisciplinary follow-up and treatment, he was discharged on November 8, 2023, showing a positive response and improved quality of life. This case highlights the importance of a comprehensive approach in pemphigus vulgaris treatment and the need for ongoing monitoring to prevent relapses and future complications.

Rituximab – An effective first line treatment in pemphigus vulgaris patients

: Rituximab is a humanized chimeric anti-CD20 monoclonal antibody that quickly lowers blood serum levels of anti-desmoglein autoantibodies, causing pemphigus lesions to heal. To evaluate the efficacy of Rituximab in treating pemphigus vulgaris. : We identified and included 14 patients (3 males and 11 females), in the age group of 14 years to 67 years, of severe pemphigus vulgaris from 2016 to 2022 treated at our institution with rituximab infusion in our retrospective case study. Baseline anti-desmoglein antibodies 1 and 3 levels were elevated in all the patients. 12 of these patients received rituximab infusion as first-line treatment. 11(out of 14) patients received two doses of rituximab (1 gram in each) as intravenous infusions, as per the rheumatoid arthritis protocol; whereas, 3 (out of 14) received a single dose of intravenous rituximab infusion (1 gram). These patients were kept on maintenance dose of prednisolone/ mycophenolate mofetil post rituximab infusion. : 14 patients of pemphigus vulgaris were enrolled in the study. The follow-up period ranged from 2 months to 6 years. All 14 patients responded well to therapy. No serious adverse events were observed. 10 (71.4%) of the 14 patients exhibited complete remission; whereas, 4 (28.6%) showed partial remission. Those with partial remission experienced relapse but the subsequent episode was less severe than the first episode. However, 2 patients who were non-responders to high doses of systemic steroids expired. : For treating patients with pemphigus vulgaris, rituximab infusion was found to be safe and effective. Hence, it should be considered as first-line for management of pemphigus vulgaris.

The efficacy and safety of low-dose rituximab in the treatment of pemphigus vulgaris: a cohort study

Background Rituximab (RTX) is considered the first-line treatment for pemphigus vulgaris (PV), which is a B-cell-mediated acquired autoimmune disease. However, no consensus on the optimum dosage has been achieved. Objectives To investigate the efficacy and safety of low-dose RTX (a single infusion of 500 mg) for the treatment of PV, a cohort study was conducted for patients with PV, along with a 12-month follow-up following the administration of RTX. Methods Patients with moderate or severe PV were divided into group A (low-dose RTX combined with corticosteroids) and group B (corticosteroids alone). Data on complete remission (CR) rates, doses of corticosteroids, cumulative doses of corticosteroids at the third, sixth, and twelfth months, pemphigus disease area index and adverse effects (AEs) were collected. Results Forty-four patients with moderate or severe PV were enrolled in this study (19 in group A and 25 in group B). Patients treated with low-dose RTX had higher CR rates, lower doses of corticosteroids at the third, sixth, and twelfth months, lower cumulative doses of corticosteroids at the sixth and twelfth months, and fewer AEs than those who received corticosteroids alone. Conclusions This study indicated that low-dose RTX may be a beneficial and secure therapy option for patients with moderate to severe PV.

Impact of Rituximab in the Management of Pemphigus Vulgaris: A Case Report

The term “Pemphigus” is derived from the Greek word Pemphix means “bubble or blister” and Vulgaris means “common”. It is a potentially life-threatening disease that causes erosions and blisters of the skin and mucous membranes. It is a chronic, rare, intra-epidermal bullous disease with a potentially fatal outcome and was originally named by Wickman in the years 1791. Pemphigus is an uncommon disease with an incidence rate ranging from 0.5 to 3.2 per 100,000 per year. In this article, we discussed a rare case report on the impact of Rituximab in the management of Pemphigus Vulgaris. The Inj. Rituximab was found to be more effective in treating Pemphigus Vulgaris along with supportive medication. By considering different literature, it has been concluded that Pemphigus Vulgaris has a relapse in nature. So, ongoing monitoring and adjustment of treatment are often necessary to manage the condition. Patient must require regular follow-up appointments with his healthcare professional to monitor the disease actively and adjust the treatment as necessary depending upon their clinical manifestations.

Trichoscopy of pemphigus vulgaris in remission. A tool for treatment-cessation decision making. Preliminary results

Background: One of the controversies in the treatment of Pemphigus Vulgaris (PV), is the time of treatment cessation. Scalp involvement is often the most stubborn to treatment human area and may reflect disease activity. Trichoscopy (Dermoscopy of hair and scalp) features have not been studied in clinically “healthy-looking” skin of PV patients in full remission. Aims and Objectives:To find out if there are any trichoscopy features in PV patients in clinical and immunologic remission, and accommodate treatment decision. Methods: The age and disease duration of 3 women with PV were correspondingly 62, 63, 55 and 5, 6, 15 years. Treatment at examination was 2,5 mg prednisone(Pr), for the last 22, 5 and 7 months. The 2nd patient was also taking 1 mg/kg/d of Azathioprine. Anti Desmoglein antibodies turned to negative since Oct/2017, Feb/2017, Sept/2015. Contact dermoscopy and hand lens X10 magnifier for clinical evaluation, were used. Results: A red hue background, peripillar scales and tubular casts were observed by trichoscopy in the first 2 patients. Only peripilar scales on a small brown round area were the features in the 3rd patient. Treatment cessation was decided only in this patient, since casts observed in the other two patients, were considered as a consequence of outer root sheath acantholysis and a sign of disease activity. Patients’ follow up justified the aforementioned treatment decisions. Conclusions: Areas of red hue skin background and movable cylindical scales (casts) around hair shafts may be observed, even in the absence of apparent scalp clinical involvement, in PV patients in remission. Since these features may drive physicians to treatment continuation, dermoscopy of the scalp is required in clinically “healthy-looking” PV patients, before a decision of treatment cessation is made. Future studies with greater number of patients, furtherly validated by long term disease course outcome, are needed.

A case report on generalized pemphigus vulgaris treated with rituximaba

Background: Pemphigus vulgaris has an obscure etiology; the presence of autoantibodies is coherent with an autoimmune disease. Rituximab a monoclonal antibody that specifically targets the CD20 antigen of B lymphocytes, has arisen as a novel treatment approach for pemphigus vulgaris. Case presentation: A 39-year-old male patient presented with a three-month history of mouth ulcers, poor oral hygiene accompanied with heavy tobacco smoking and alcohol consumption. He was diagnosed with pemphigus vulgaris. The disease gradually progressed to involve other body parts. The patient had shown partial improvement after conventional therapy (oral cefuroxime, oral prednisolone with azathioprine) and was later on successfully treated with rituximab. After 90 days of follow-up, no future recurrence was observed. Conclusion: With this case, the authors would like to aware other clinicians of the potential use of rituximab in treating pemphigus vulgaris, especially when the conventional therapy fails.

SARS-CoV-2 Vaccination Effectiveness in Rituximab-Treated Patients Affected by Pemphigus Vulgaris

SARS-CoV-2 Vaccination Effectiveness in Rituximab-Treated Patients Affected by Pemphigus Vulgaris

Use of rituximab in the treatment of pemphigus vulgaris in a juvenile patient

Use of rituximab in the treatment of pemphigus vulgaris in a juvenile patient

CO120 Evidence Gap Analysis of the Burden of Illness and Treatment of Pemphigus Vulgaris and Foliaceus

To identify evidence gaps in the literature of the burden of illness and treatment of pemphigus vulgaris (PV) and pemphigus foliaceus (PF) to support the launch of efgartigimod to treat these rare diseases.

Role of Rituximab in the Treatment of Pemphigus Vulgaris: Patient Selection and Acceptability

Anti-CD20 monoclonal antibody rituximab is an approved adjuvant treatment, in combination with oral corticosteroids, for patients with pemphigus vulgaris, a severe and potentially life-threatening autoimmune blistering skin disorder. Updated approaches to the management of pemphigus vulgaris support rituximab as a first-line adjuvant treatment to induce remission early in the course of disease; however, its feasibility in the clinical setting is often reduced by a series of limitations, including high cost of this biological drug, physician and patient concern for the risk of adverse reactions, and uncertainty regarding the optimum dosing and schedule of administration. The standard approved rituximab dosages, which are derived from lymphoma protocols, have been recognized to exceed the effective dose required for inducing B cell depletion, since the B cell burden in pemphigus vulgaris is much lower than in lymphoproliferative disorders. To overcome these limitations, recent research has investigated alternative regimens of rituximab, using lower doses of the drug. Moreover, differences in patient and disease characteristics that are highlighted in the literature strongly suggest that therapy should be tailored individually on a case-by-case basis: personalized treatment schedules may be necessary to optimize response to treatment and tolerability in different subjects, with the possibility of repeated infusions for severe forms and in case of relapse. Finally, low-dose regimens of rituximab were suggested to be favorable during the COVID-19 pandemic by providing a lesser degree of immune cell depletion while retaining a sufficient response. In conclusion, the current literature suggests that lower-dose regimens of rituximab are not only tolerable and cost-effective but may also be associated with a positive response in pemphigus vulgaris, comparable to that achieved with higher doses especially in early disease. Further evidence from rigorous clinical trials will be required to optimize lower-dose regimens of RTX and establish their position within the treatment scenario of pemphigus vulgaris.

Trichoscopy of pemphigus vulgaris in remission. A tool for treatment-cessation decision making? Preliminary results

Background One of the controversies in the treatment of Pemphigus Vulgaris (PV), is the time of treatment cessation. Scalp involvement is often the most stubborn to treatment body site and may reflect disease activity. Trichoscopy (Dermoscopy of scalp) features have not been studied in clinically “healthy-looking” skin of patients in full remission. Aims and Objectives To find out if there are any trichoscopy features in PV patients in clinical and immunologic remission, and accommodate treatment decision Methods The age and disease duration of 3 women with PV were correspondingly 62, 63, 55 and 5, 6, 15 years. Treatment at examination was 2,5 mg prednisone(Pr), for the last 22, 5 and 7 months. The 2nd patient was also taking 1 mg/kg/d of Azathioprine. Anti Desmoglein antibodies turned to negative since Oct/2017, Feb/2017, Sept/2015. Contact dermoscopy and hand lens X10 magnifier for clinical evaluation, were used. Results A red hue background, peripillar scales and tubular casts were observed by trichoscopy in the first 2 patients. Only peripilar scales on a small brown round area were the features in the 3rd patient. Treatment cessation was decided only in this patient, since casts observed in the other two patients, were considered as a consequence of outer root sheath acantholysis and a sign of disease activity Conclusions Areas of red hue skin background and movable cylindical scales (casts) around hair shafts may be observed, even in the absence of apparent scalp clinical involvement, in PV patients in remission. Since these features may drive physicians to treatment continuation, dermoscopy of the scalp is required in clinically “healthy-looking” PV patients, before a decision of treatment cessation is made. Future studies with greater number of patients, furtherly validated by long term disease course outcome, are needed

Effective Management of Pemphigus Vulgaris: A Case Report

Pemphigus vulgaris is a unique chronic condition of bullous autoimmune dermatosis whose development and diagnosis is mostly unidentifiable. It is classified as a specific hypersensitivity type II reaction that initiates the antibodies against the desmosomes, which are the maculae adherentes of the skin also known as the adhering spots in common term. These adhering spots keep the certain layers and compartments of the skin intact. When the desmosomes are invaded by the antibodies, the intact layers of the skin separate out and the clinical picture resembles a blister. With the passage of time the condition becomes worse and without treatment and monitoring the blister type lesions increase in size and surface area of the body similar to the condition of severe burn. The prime focus in the treatment of pemphigus vulgaris is to control the infirmity, prevent relapses, avoid adverse events and complications associated with the broad use of the steroids and immunosuppressive agents. Systemic corticosteroids evidenced as the golden standard in the treatment regimens of the pemphigus vulgaris. Azathioprine and mycophenolate mofetil are considered as the choice of drug in the treatment of the pemphigus vulgaris as a steroid sparing agent. Rituximab is believed to be immensely efficacious in unmanageable and refracted types of pemphigus vulgaris when other remedies fail to control the disease. One of the most noticeable complications of the pemphigus vulgaris is the compromised quality of life due to the blister forming all over the body which aggravates the soreness and pain on the affected area leading to ulceration and formation of erosive lesions. If there is a recurrence of the disease then the steroids doses will be checked properly and if needed then the doses could also be increased according to the weight in safe limit thus augmenting the immunosuppressants in the body and find an alternate safe drug in concomitant therapy

A randomised clinical trial to assess the adjuvant potential of methotrexate to corticosteroids in mucosal or limited mucocutaneous pemphigus vulgaris

Glucocorticoids are the mainstay of treatment for pemphigus vulgaris (PV). However, the requirement of high doses for long durations often leads to serious adverse events. Methotrexate as an adjuvant has shown potential in retrospective studies but randomized trials are lacking. The objective of the study was to assess the adjuvant potential of methotrexate in mucosal/limited mucocutaneous PV. In this randomised prospective study, 44 patients with mucosal/limited mucocutaneous PV were randomised (1:1) to receive either prednisolone 1 mg/kg/day (later fixed at a maximum dose of 60 mg/day) alone or with methotrexate 0.3 mg/kg/week for 9-months study period. Prednisolone dose was tapered once there was an 80% reduction in Pemphigus Disease Activity Index. Outcome measures were total cumulative dose of prednisolone, the proportion of patients achieving disease control, time taken for disease control and remission on minimal treatment, and adverse effects. No significant difference in the total cumulative dose of prednisolone among the groups was observed (p = 0.68). Disease control was achieved in 95.5% and 86.4% of patients in the prednisolone alone group, and prednisolone and methotrexate group respectively (p = 0.61). No statistically significant difference was observed among the groups with respect to the proportion of patients achieving remission, time taken for disease control and remission, and the number of adverse events. Our study showed no additional benefit of methotrexate to prednisolone in the treatment of mucosal/limited mucocutaneous PV.Trial registration: CTRI/2018/07/015002; Registered on 23/07/2018]; Trial Registered Retrospectively. http://ctri.nic.in/Clinicaltrials/pdf_generate.php?trialid=24964&EncHid=&modid=&compid=%27,%2724964det%27.

Effect of NUDT15 polymorphisms on early hematological safety of low-dose azathioprine in Chinese patients with pemphigus vulgaris: A prospective cohort study.

Azathioprine (AZA) is the preferred immunosuppressant for treating pemphigus vulgaris (PV), with discontinuation mainly attributed to hematological adverse events (AE). Reportedly, nucleoside diphosphate‐linked moiety X‐type motif 15 (NUDT15) polymorphisms have been strongly associated with thiopurine‐induced leukopenia. To investigate hematological AE of low‐dose AZA based on NUDT15 genotypes among patients with PV, a prospective cohort study was conducted in patients with PV, followed‐up for the first 8 weeks after AZA administration. All patients were divided into wild homozygous and heterozygous NUDT15 groups. Both groups initiated AZA at low dose (50 mg/day) and continued with different dose‐escalating approaches. Bone marrow suppression was considered the principal outcome. Overall, 62 patients with PV were enrolled (48 in the wild homozygous NUDT15 group vs. 14 in the heterozygous NUDT15 group). Except for median maintenance doses of AZA, no statistically significant differences were observed between the two groups in terms of age, sex, white blood cells, neutrophil count, platelet count, hemoglobin level, median final doses of corticosteroids (mg prednisone equivalent), pemphigus disease area index, and anti‐desmoglein 1/3 autoantibodies. In both groups, patients presented similar hematological AE and treatment responses after administration of different low‐dose AZA treatment strategies. Low‐dose AZA based on NUDT15 genotypes can reduce the risk of early hematological AE among patients with PV.

Dural venous sinus thrombosis: A rare complication of pulse corticosteroid therapy in pemphigus vulgaris

Systemic pulse corticosteroid therapy is used widely in the treatment of pemphigus vulgaris. Dural venous sinus thrombosis as a complication of pulse therapy has not been reported in the literature. A middle-aged female with pemphigus vulgaris was started on monthly pulse dexamethasone therapy with daily azathioprine as an adjuvant. After two pulse therapies, she developed throbbing headache, which on further evaluation was determined to be due to dural venous thrombosis. Other causes of dural venous thrombosis were excluded. Pulse therapy was stopped and other medications were started. The headache subsided within two weeks of stopping pulse therapy. Corticosteroids may play the role of a procoagulant in producing cerebral venous sinus thrombosis. Herein, we report a rare case of dural venous sinus thrombosis due to pulse steroid therapy in pemphigus vulgaris.

LB773 Treating pemphigus vulgaris (PV) and foliaceus (PF) by inhibiting the neonatal Fc receptor: Phase 2 multicentre open-label trial with efgartigimod

Efgartigimod, an engineered Fc fragment that inhibits the activity of the neonatal Fc receptor (FcRn), was evaluated in an open-label phase 2 adaptive trial (NCT03334058). Thirty-four mild to moderate PV or PF patients were enrolled to evaluate the safety, pharmacodynamics, pharmacokinetics, and efficacy of efgartigimod. In four sequential cohorts, efgartigimod was dosed at 10 or 25 mg/kg intravenously with various dosing frequencies, as monotherapy or add-on therapy to low-dose oral prednisone.

Successful treatment of pemphigus vulgaris in children using methylprednisolone pulsed treatment

Pemphigus vulgaris (PV) is a disease which clinical manifestation of mucosal and skin surface blisters classified as autoimmune disease. Intermittent adhesion damage to the epidermis due to IgG autoantibodies complements and sometimes against Dsg-1 and Dsg-3 can cause acantholysis. PV mostly found in Jewish, Mediterranean descent, and Asians. PV most commonly appeared in elderly beteween 40 and 70 years old. Methylprednisolone pulsed-dose was known as an effective therapy for PV in adult. This case shows successful methylprednisolone pulsed-dose therapy in childhood PV.

The evolving role of rituximab in the treatment of pemphigus vulgaris: a comprehensive state-of-the-art review

ABSTRACT Introduction Pemphigus vulgaris (PV) is a life-threatening autoimmune mucocutaneous blistering disease. Systemic corticosteroids (CS), while life-saving, have several serious side effects. To improve treatment and prognosis, recently rituximab (RTX), a chimeric monoclonal antibody against CD20 molecule on B cells, has become popular. This Expert Opinion discusses clinical and scientifically relevant aspects of RTX treating PV. Area Covered This presentation describes the mechanism of action, clinical efficacy, safety, adverse events, protocols used, and clinical outcomes. Concerns for infection, reactivation of latent or previous infections, and high relapse rate are discussed. Expert opinion Use of RTX in PV is still a work in progress. There are many unanswered questions. FDA did not provide a protocol or guidelines. Whenever RTX is used, systemic corticosteroids are simultaneously used, albeit for a shorter duration and lower dose. Used in these doses for these durations they can cause immunosuppression. Would it be more appropriate if instead of ‘First Line Therapy’ it would be more advisable to use the term ‘First Adjunctive Immunosuppressive Agent’?

Efgartigimod alfa. Neonatal Fc receptor (FcRn) antagonist, Treatment of generalized myasthenia gravis, Treatment of primary immune thrombocytopenia, Treatment of pemphigus vulgaris and foliaceous, Treatment of chronic inflammatory demyelinating polyneuropathy

Efgartigimod alfa. Neonatal Fc receptor (FcRn) antagonist, Treatment of generalized myasthenia gravis, Treatment of primary immune thrombocytopenia, Treatment of pemphigus vulgaris and foliaceous, Treatment of chronic inflammatory demyelinating polyneuropathy

Can rituximab be used in the treatment of pemphigus vulgaris during the COVID-19 pandemic?

Rituximab is a monoclonal antibody that targets CD20, a B‐lymphocyte antigen; that leads to a decline in the B‐cell counts for at least a year. The patients who have received rituximab treatment in the previous 5 years with the diagnosis of pemphigus group of diseases at Cerrahpaşa Medical Faculty were questioned for COVID‐19 infection. A total of 48 patients were included in this study; only one male patient had COVID‐19 infection which had a mild course. There is no significant difference in the total number of lymphocytes between patients who have received rituximab within the previous 5 years or last year. The number of lymphocytes is independent of the number of courses of rituximab treatment received. Therefore, we suggest that all pemphigus patients who have received rituximab treatment within the previous 5 years should be careful of the preventive measures against the COVID‐19 infection irrespective of the number of treatment courses or the number of years which has passed since the treatment. The disease course was mild in the only infected patient. Thus, rituximab may be used in the treatment of pemphigus vulgaris during the COVID‐19 pandemic if its use is necessary.