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pemphigus rituximab

Marta Wieczorek, Annette Czernik
Paraneoplastic pemphigus (PNP) is a fatal autoimmune blistering disease associated with an underlying malignancy. It is a newly recognized blistering disease, which was first recognized in 1990 by Dr Anhalt who described an atypical pemphigus with associated neoplasia. In 2001, Nguyen proposed the term paraneoplastic autoimmune multiorgan syndrome because of the recognition that the condition affects multiple organ systems. PNP presents most frequently between 45 and 70 years old, but it also occurs in children and adolescents...
2016: Clinical, Cosmetic and Investigational Dermatology
K Gleghorn, J Wilson, M Wilkerson
Rituximab is an anti-CD20 monoclonal antibody with considerable potential in dermatology due to an increase in off-label indications. Chronic graft-versus-host disease and pemphigus vulgaris are two of the most promising indications for off-label use of rituximab. It is a generally safe alternative that should be considered when traditional therapy with corticosteroids or immunosuppressants has failed or caused significant intolerance. Currently, rituximab is only FDA-approved for treatment of follicular and diffuse large B-cell non-Hodgkin's lymphoma, rheumatoid arthritis, chronic lymphocytic leukemia, granulomatosis with polyangiitis (formerly Wegener's granulomatosis) and microscopic polyangiitis...
September 2016: Skin Therapy Letter
Rajsmita Bhattacharjee, Dipankar De, Sanjeev Handa, Ranjana W Minz, Biman Saikia, Neha Joshi
BACKGROUND: Robust evidence for the efficacy of rituximab monotherapy in pemphigus is lacking. The effects of rituximab on T-regulatory cells (Tregs) in pemphigus have not been studied. OBJECTIVE: The primary objective was to assess the efficacy of rituximab monotherapy in severe pemphigus vulgaris. The secondary objectives were to assess whether counts of different subsets of Tregs in the peripheral blood correlate with baseline clinical severity and whether clinical response in severe pemphigus is associated with an alteration in the Treg count...
September 1, 2016: Dermatology: International Journal for Clinical and Investigative Dermatology
Lifang Wang, Hui Deng, Mei Mao
Paraneoplastic pemphigus (PNP) is an autoimmune blistering disease associated with neoplasms. The disease is most commonly of lymphoproliferative origin and presents high mortality. We describe a patient with PNP and myasthenia gravis associated with inflammatory pseudotumor-like follicular dendritic cell sarcoma, as well as the response to rituximab.
August 2016: Clinical Case Reports
Vinod Kumar Sharma, Neetu Bhari, Somesh Gupta, Kanika Sahni, Neena Khanna, M Ramam, G Sethuraman
BACKGROUND: Pulsed corticosteroids have been used successfully for the management of pemphigus. However, prolonged use of glucocorticoids may be associated with adverse effects and some patients show a poor response to conventional therapy. Biologics have shown a promising role in such cases; however, there is limited data from the Indian subcontinent. OBJECTIVE: The primary objective was to assess the efficacy and adverse effects of rituximab in pemphigus. The secondary objective was to measure the cumulative doses of corticosteroids required for these patients...
July 2016: Indian Journal of Dermatology, Venereology and Leprology
D T Greenblatt, E C Benton, R W Groves, J F Setterfield
Pemphigus vulgaris (PV) is an autoimmune blistering disease affecting the skin and mucous membranes. Rituximab, a CD20 chimeric monoclonal antibody, has efficacy in PV management. We report a case of severe oral PV that showed a progressive response to repeated courses of rituximab, culminating in a rapid response within 4 weeks following severe relapse 4 years after initial therapy. It demonstrates the progressively shorter time to achieve partial or complete remission following rituximab infusions, combined with minimal adjuvant therapy over a 7-year follow-up period...
July 2016: Clinical and Experimental Dermatology
Paul R J Ames, Maria Graf, Fabrizio Gentile
BACKGROUND: Several autoimmune skin disorders are characterised by an increased risk of thrombosis, with bollous pemphigoid carrying a higher risk than pemphigus vulgaris (PV). We describe the case of a middle aged gentleman who developed recurrent venous thromboembolism despite adequate oral anticoagulation during very active PV that required escalation of treatment to bring the disease under control. CASE PRESENTATION: In May 2014 a 49 year gentleman was admitted for widespread mucocutaneous blistering diagnosed as PV by histology and immunofluorescence...
2016: Thrombosis Journal
A Razzaque Ahmed, Tegan Nguyen, Srini Kaveri, Zachary S Spigelman
BACKGROUND: Conventional therapy for pemphigus vulgaris (PV) consists of high-dose systemic corticosteroids (CS) and immunosuppressive agents (ISA). This combination may be ineffective, cause serious adverse events or relapses in some patients. OBJECTIVE: To determine if the combination of intravenous immunoglobulin (IVIg) therapy and rituximab (RTX) can be used as first-line therapy in PV patients in whom systemic CS and ISA are contraindicated and evaluate its ability to produce long-term sustained remissions...
May 2016: International Immunopharmacology
Vinod Kumar Sharma, Neetu Bhari, Somesh Gupta, Kanika Sahni, Neena Khanna, M Ramam, G Sethuraman
BACKGROUND: Pulsed corticosteroids have been used successfully for the management of pemphigus. However, prolonged use of glucocorticoids may be associated with adverse effects and some patients show a poor response to conventional therapy. Biologics have shown a promising role in such cases; however, there is limited data from the Indian subcontinent. OBJECTIVE: The primary objective was to assess the efficacy and adverse effects of rituximab in pemphigus. The secondary objective was to measure the cumulative doses of corticosteroids required for these patients...
February 9, 2016: Indian Journal of Dermatology, Venereology and Leprology
F Galimberti, A P Fernandez
The anti-CD20 peripheral B-cell depleting monoclonal antibody, rituximab, has been shown to be a safe and effective treatment for refractory pemphigus vulgaris (PV), a potentially fatal autoimmune blistering disease. We report a patient who developed skin nodules and arthralgias following successful treatment of refractory PV with rituximab. Clinical, serological and histological findings were consistent with a diagnosis of sarcoidosis. The nodules promptly responded to treatment with corticosteroids, and resolved without recurrence when the medication was tapered several months later...
June 2016: Clinical and Experimental Dermatology
Amy Huang, Raman K Madan, Jacob Levitt
The conventional treatment for patients with pemphigus vulgaris (PV) centers on global immunosuppression, such as the use of steroids and other immunosuppressive drugs, to decrease titers of antidesmoglein autoantibodies responsible for the acantholytic blisters. Global immunosuppressants, however, cause serious side effects. The emergence of anti-CD20 biologic medications, such as rituximab, as an adjunct to conventional therapy has shifted the focus to targeted destruction of autoimmune B cells. Next-generation biologic medications with improved modes of delivery, pharmacology, and side effect profiles are constantly being developed, adding to the diversity of options for PV treatment...
April 2016: Journal of the American Academy of Dermatology
Leah Kincaid, Miriam Weinstein
Pediatric pemphigus vulgaris (PV) is a rare autoimmune bullous dermatosis that poses a diagnostic and therapeutic challenge. Rituximab, an anti-CD20 monoclonal antibody, is an important medication in adult PV but has rarely been used to treat pediatric disease. We describe successful rituximab therapy in a 4-year-old, the youngest patient with PV ever reported to receive rituximab. A live attenuated vaccination was later given without incident. We also review rituximab clinical outcomes, toxicity, dosing protocols, and relapse in children with PV...
March 2016: Pediatric Dermatology
A Razzaque Ahmed, Srini Kaveri, Zachary Spigelman
No abstract text is available yet for this article.
December 31, 2015: New England Journal of Medicine
Fernanda Bellodi-Schmidt, Kara N Shah
Dermatologists have witnessed the increasing availability of novel biologic response modifiers for the treatment of inflammatory and autoimmune diseases in recent years. The most common dermatologic indication for the use of biologic response modifiers in adults is psoriasis, but the U.S. Food and Drug Administration has not approved any of these agents for use in any dermatologic disease in children with the exception of omalizumab, and as such, use in this population is considered off-label. In this review, we focus on the use of these agents in children to treat inflammatory skin diseases other than psoriasis, including atopic dermatitis, hidradenitis suppurativa, pemphigus vulgaris, bullous pemphigoid, and toxic epidermal necrolysis, with an emphasis on the use of etanercept, infliximab, rituximab, omalizumab, and ustekinumab...
January 2016: Pediatric Dermatology
Anna Sinistro, Valentina Calabresi, Francesca Lupi, Francesco Sera, Alessandra Frezzolini, Marina Ruffelli, Ornella De Pità, Diana Camaioni, Giuseppe Cianchini, Giovanni Di Zenzo
BACKGROUND: Pemphigus vulgaris (PV) is an autoimmune blistering disease mediated by IgG autoantibodies targeting desmogleins (Dsgs). The anti-CD20 monoclonal antibody rituximab is increasingly used in corticosteroid-resistant PV patients. In a subset of rituximab-treated patients in remission, high ELISA index values have been reported; however, their significance remains so far unclear. OBJECTIVE: To address the discrepancy between anti-Dsg3 serum antibody titers and disease severity...
November 2015: European Journal of Dermatology: EJD
Stamatis Gregoriou, Ourania Efthymiou, Christina Stefanaki, Dimitris Rigopoulos
The main objective in the treatment of pemphigus vulgaris is to control the disease, prevent relapses, and avoid adverse events associated with the prolonged use of steroids and immunosuppressive agents. Systemic corticosteroids remain the gold standard treatment for pemphigus vulgaris. Azathioprine and mycophenolate mofetil are the first line of steroid-sparing treatment. Rituximab is extremely effective in recalcitrant pemphigus, when other treatments fail to control the disease. The European Dermatology Forum recommends tapering prednisolone by 25% every 2 weeks after the consolidation phase, and a 5 mg reduction every 4 weeks when the dose is reduced to <20 mg...
2015: Clinical, Cosmetic and Investigational Dermatology
Chika Namba, Mikiko Tohyama, Yasushi Hanakawa, Masamoto Murakami, Yuji Shirakata, Takuya Matsumoto, Koichiro Suemori, Norito Ishii, Takashi Hashimoto, Koji Sayama
Paraneoplastic pemphigus (PNP) is an autoimmune blistering disease that presents as severe mucosal erosions and variable cutaneous lesions and is primarily associated with hematologically malignant or benign diseases. A 59-year-old Japanese woman presented with oral, ocular and vaginal mucosal erosions and erythema as well as blistering on her trunk and limbs. She developed bronchiolitis obliterans; lymphadenopathy in the cervical, subclavian, para-aortic and intraperitoneal regions; and splenomegaly. PNP with B-cell lymphoma was diagnosed...
April 2016: Journal of Dermatology
Taichi Hirano, Yusuke Higuchi, Hiromichi Yuki, Shinya Hirata, Kisato Nosaka, Norito Ishii, Takashi Hashimoto, Hiroaki Mitsuya, Yutaka Okuno
A 60-year-old male patient suffered from mild exertional dyspnea, wheezing, and systemic blisters. He was diagnosed with paraneoplastic pemphigus (PNP) with follicular lymphoma in the pancreas head and pelvic cavity. He was first treated with eight cycles of rituximab; his blisters and erosions gradually improved and highly elevated levels of auto-antibodies related to PNP gradually decreased to normal levels. However, obstructive and restrictive respiratory failure still progressed. Computed tomography of the inspiratory and expiratory phases revealed obstructive pulmonary disorder, leading to a diagnosis of bronchiolitis obliterans (BO)...
2015: Journal of Clinical and Experimental Hematopathology: JCEH
Pedram Noormohammadpour, Amirhooshang Ehsani, Hossein Mortazavi, Maryam Daneshpazhooh, Kamran Balighi, Mohammad Mofidi, Fatemeh Gholamali, Ali Sadeghinia
Pemphigus is a severe life-threatening blistering disease associated with autoantibodies against cell adhesion proteins desmogleins 1 and 3. Patients with severe pemphigus commonly show high rates of relapse after conventional immunosuppressive therapy. The newly developed drug Rituximab showed impressing promises in the treatment of refractory pemphigus vulgaris (PV). In the present study the efficacy of a single course rituximab therapy in the treatment of PV was investigated. Eighteen patients with severe recalcitrant PV were recruited to this study...
2015: EXCLI journal
Yan Ling Kong, Yen Loo Lim, Nisha Suyien Chandran
BACKGROUND: Autoimmune blistering diseases (AIBDs) are rare in children and their prevalence in Singapore is unclear. We aimed to investigate the clinical and immunopathologic characteristics of children diagnosed with AIBDs in Singapore. MATERIALS AND METHODS: The clinical and histology databases at the National Skin Centre in Singapore were searched to identify patients younger than 18 years old diagnosed with an AIBD from January 1, 1998, through December 31, 2012...
November 2015: Pediatric Dermatology
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