Pemphigus vulgaris - approach and management.

Pemphigus vulgaris - approach and management. Exp Ther Med. 2019 Dec;18(6):5056-5060 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 pathophysiology of these dermatoses is very well understood, complemented by recent genetic studies. The gold standard for the diagnosis of pemphigus vulgaris is the detection of autoantibodies or complement component 3 by direct immunofluorescence microscopy of a perilesional biopsy. Early diagnosis and initiation of treatment are necessary in order to achieve a favorable prognosis. Although the first line of treatment is corticotherapy, there are no clear guidelines on dosing regimens, and long-term adverse effects are important. Corticosteroid-sparing adjuvant therapies have been employed in the treatment of PV, aiming to reduce the necessary cumulative dose of corticosteroids. In addition, therapies with anti-CD20 antibodies are used, but antigen-specific immune supp...
Source: Experimental and Therapeutic Medicine - Category: General Medicine Tags: Exp Ther Med Source Type: research

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Zhou and colleagues detected ectopic lymphoid structures (ELS) that resemble tertiary lymphoid organs in pemphigus vulgaris and pemphigus foliaceous lesions. ELS were characterized by B cells reactive to the desmoglein 3 autoantigen, and were associated with active disease and B cell expansion. B cell differentiation in ELS was supported by the detection of centroblasts, plasmablasts, and plasma cells in these lesions. Chemokines that may induce B cell migration to pemphigus lesions were also detected in ELS.
Source: Journal of Investigative Dermatology - Category: Dermatology Tags: Editorial Source Type: research
Pemphigus is an autoimmune bullous disease characterized by IgG production against desmogleins. The major sites of autoantibody production are thought to be lymph nodes, spleen, and bone marrow. Previously, it has been suggested that autoreactive B cells might exist in the skin lesions in pemphigus and produce autoantibodies. In their report, Zhou et  al. expanded their previous studies and reported that ectopic lymphoid-like structures were found in pemphigus skin lesions, wherein B-cell differentiation and lesional B-cell expansion might progress.
Source: Journal of Investigative Dermatology - Category: Dermatology Authors: Tags: Commentary Source Type: research
Pemphigus is a chronic autoimmune blistering disorder, characterized by (muco-)cutaneous erosions due to autoantibodies against desmoglein 3 and/or 1. Pemphigus induction might be associated with drugs, malignancy or radiation therapy (RT); the latter being only rarely described. A rigorous literature review revealed around 30 cases of RT-associated pemphigus, which had been primarily treated with topical and/or systemic steroids, in some cases also dapsone or few other immunosuppressive agents were given. The most common underlying cancer type was breast cancer. We here present a 63-year-old male patient, who was pre-trea...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
Publication date: Available online 18 January 2020Source: Gynecologic Oncology ReportsAuthor(s): Bijan Morshedi, Kari RingAbstractPemphigus is a group of autoimmune intraepidermal blistering diseases caused by immunoglobulins directed against keratinocyte cell surface components. In this case report, we identify a non-classical paraneoplastic pemphigus (PNP) foliaceous related to an undifferentiated uterine sarcoma.The patient is a 54-year-old female with a past medical history of arthritis who presented with worsening fatigue in November 2017 and an itchy, blistering, erythematous annular plaque that first appeared on her...
Source: Gynecologic Oncology Reports - Category: OBGYN Source Type: research
Source: Journal of the American Academy of Dermatology - Category: Dermatology Authors: Source Type: research
Authors: Pires CAA, Einecke YS, Pereira NG, Costa CCC, Cunha ACDS, Moreira AG, Rodrigues FDN, Xavier MB Abstract Background: Pemphigus are rare chronic autoimmune blistering disorders with challenging therapeutic management.Objective: To investigate the therapeutic outcomes of pemphigus patients admitted to a dermatology referral service in northern Brazil.Materials and methods: We conducted a retrospective analysis of 32 patients with histopathological diagnosis of pemphigus between 2010 and 2016. Clinical and epidemiological data were collected. Therapeutic outcome was evaluated according to the criteria proposed...
Source: Journal of Dermatological Treatment - Category: Dermatology Tags: J Dermatolog Treat Source Type: research
CONCLUSION: This survey of the practical use of RTX confirms its interest in moderate to severe pemphigus as a second-line or subsequent treatment, a situation that probably remains relevant even if this molecule is increasingly used as first-line therapy. PMID: 31955970 [PubMed - as supplied by publisher]
Source: Annales de Dermatologie et de Cenereologie - Category: Dermatology Authors: Tags: Ann Dermatol Venereol Source Type: research
Cristina Sousa, Manuel Campos, Jorge Lopes, Armando Baptista, Ana Oliveira
Source: Immunome Research - Category: Allergy & Immunology Source Type: research
Source: Journal of the American Academy of Dermatology - Category: Dermatology Authors: Source Type: research
Sairafi D Abstract Hailey-Hailey disease (HHD), or familial benign pemphigus, is a rare autosomal dominant acantholytic dermatosis characterized by blistering, and erythematous, erosive and macerated plaques located primarily in intertriginous areas. Secondary bacterial and viral infections are common. There is no "gold standard" treatment, although many therapeutic modalities have been proposed. PMID: 31912486 [PubMed - as supplied by publisher]
Source: The British Journal of Dermatology - Category: Dermatology Authors: Tags: Br J Dermatol Source Type: research
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