Anterior scleritis in a patient of pemphigus vulgaris while on immunosuppressive treatment

Mihika Dube, Brijesh Takkar, Dinesh P Asati, Sourabh JainIndian Journal of Dermatology 2019 64(6):499-500
Source: Indian Journal of Dermatology - Category: Dermatology Authors: Source Type: research

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AbstractRituximab (MabThera®, Rituxan®), a chimeric murine/human anti-CD20 monoclonal antibody administered by intravenous infusion, is indicated for the treatment of moderate to severe pemphigus vulgaris (PV), in combination with a tapering course of corticosteroids. Approval in the EU and USA was based on data for the subset of patients with newly-diagnosed, previously untreated PV participating in the randomized, controlled RITUX 3 study; rituximab plus short-course prednisone resulted in a  >  3-fold higher rate of complete remission off prednisone therapy and a >  2-fold decre...
Source: American Journal of Clinical Dermatology - Category: Dermatology Source Type: research
Authors: Popescu IA, Statescu L, Vata D, Porumb-Andrese E, Patrascu AI, Grajdeanu IA, Solovastru LG Abstract The place of pemphigus vulgaris (PV) among autoimmune bullous dermatoses is well known. In pemphigus, IgG autoantibodies are directed against desmogleins 1 and 3, which are part of the cadherin family of cell-cell adhesion molecules. These structures are responsible for maintaining the intercellular adherence in stratified squamous epithelia, such as the skin and oral mucosa. The incidence of autoimmune bullous dermatoses is steadily increasing, being associated with a high degree of morbidity. The pathophys...
Source: Experimental and Therapeutic Medicine - Category: General Medicine Tags: Exp Ther Med Source Type: research
Desmosomes reinforce cohesion of epithelial cells at the interface between adjacent cells. They include the cadherin-type adhesion molecules desmoglein 1 (Dsg1) and Dsg3. Pemphigus vulgaris (PV) is an autoimmune disease in which circulating autoantibodies (PV-IgG) targeting Dsg1 and 3 cause characteristic epidermal blister formation. It has been shown that PV-IgG binding induced activation of kinases such as ERK and PKC, and inhibition of these signaling pathways prevented loss of cell cohesion in cell cultures. However, the role of Erk and PKC in blister formation and regulation of desmosome ultrastructure in human skin a...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
The clinical, histologic, and immunopathologic features of IgA pemphigus have not been studied on a large scale.
Source: Journal of the American Academy of Dermatology - Category: Dermatology Authors: Source Type: research
Source: Journal of the American Academy of Dermatology - Category: Dermatology Authors: Source Type: research
We report a case of pemphigus vegetans in an 85-year-old female presenting with hypertrophic verrucous lesions over external genitalia and perianal region. There was no history of preceding oral lesions. The diagnosis of pemphigus vegetans was considered on the clinical ground and confirmed by histopathological examination.
Source: Indian Journal of Sexually Transmitted Diseases - Category: Infectious Diseases Authors: Source Type: research
Objectives: To test the hypothesis that familial Mediterranean fever (FMF)-associated autoinflammation may exaggerate the tendency toward adaptive immunopathology or spondyloarthritis (SpA)-associated disorders including major histocompatibility complex (MHC) class I associated disorders but not classical MHC class II-associated disorders that exhibit transplacental autoimmunity including myasthenia gravis and pemphigus.Methods: Seven thousand seven hundred forty-seven FMF patients and 10,080 age- and sex-matched controls in the Clalit Health Services medical database were identified and compared in terms of prevalence of ...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
Pemphigus foliaceus is an autoimmune disease that is sporadic around the world but endemic in Brazil, where it is known as fogo selvagem (FS). Characterized by autoantibodies against the desmosomal cadherin desmoglein 1, FS causes painful erosions, and crusts that may be widespread. The recognition of antigens, including exposed sugar moieties, activates the complement system. Complement receptor 1 (CR1, CD35), which is responsible for the Knops blood group on erythrocytes (York and McCoy antigens), is also expressed by antigen-presenting cells. This regulates the complement system by removing opsonized antigens, blocking ...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
dwig RJ Abstract The autoimmune skin blistering diseases pemphigus (vulgaris and foliaceus) and bullous pemphigoid (BP) pose a high burden on affected patients. With current treatment options, induction of remission is achieved in most patients. However, prolonged immunosuppression is required to maintain remission, and treatment-related morbidity and mortality further adds to the patients' burden1,2 . Hence, development of novel therapeutic strategies that are effective and safe is highly warranted. Recently, insights into pemphigus and BP pathogenesis identified new therapeutic targets and drugs3 . PMID: 31...
Source: The British Journal of Dermatology - Category: Dermatology Authors: Tags: Br J Dermatol Source Type: research
Increasing evidence suggests that certain subpopulations of B cells can have regulatory functions. Herein, we report immunohistochemical and clinical evidence of psoriasis following B cell depletion with rituximab, administered as therapy for pemphigus vulgaris. We also mine RNA-Seq datasets to help elucidate a role for B cells in psoriasis pathophysiology, an understudied but potentially important area of further investigation.
Source: Journal of Dermatological Science - Category: Dermatology Authors: Tags: Letter to the Editor Source Type: research
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