Pemphigus

Publication date: 7–13 September 2019Source: The Lancet, Volume 394, Issue 10201Author(s): Enno Schmidt, Michael Kasperkiewicz, Pascal JolySummaryPemphigus consists of a group of rare and severe autoimmune blistering diseases mediated by pathogenic autoantibodies mainly directed against two desmosomal adhesion proteins, desmoglein (Dsg)1 and Dsg3 (also known as DG1 and DG3), which are present in the skin and surface-close mucosae. The binding of autoantibodies to Dsg proteins induces a separation of neighbouring keratinocytes, in a process known as acantholysis. The two main pemphigus variants are pemphigus vulgaris, which often originates with painful oral erosions, and pemphigus foliaceus, which is characterised by exclusive skin lesions. Pemphigus is diagnosed on the basis of either IgG or complement component 3 deposits (or both) at the keratinocyte cell membrane, detected by direct immunofluorescence microscopy of a perilesional biopsy, with serum anti-Dsg1 or anti-Dsg3 antibodies (or both) detected by ELISA. Corticosteroids are the therapeutic mainstay, which have recently been complemented by the anti-CD20 antibody rituximab in moderate and severe disease. Rituximab induces complete remission off therapy in 90% of patients, despite rapid tapering of corticosteroids, thus allowing for a major corticosteroid-sparing effect and a halved number of adverse events related to corticosteroids.
Source: The Lancet - Category: General Medicine Source Type: research

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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
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
CONCLUSIONS AND CLINICAL IMPORTANCE: BTKi monotherapy may have beneficial effects in some cases of cPF. PMID: 31899567 [PubMed - as supplied by publisher]
Source: Veterinary Dermatology - Category: Veterinary Research Authors: Tags: Vet Dermatol Source Type: research
Authors: Shahidi-Dadras M, Pishgahi M, Tabary M, Kheradmand Z, Araghi F, Dadkhahfar S, Robati RM Abstract Background: Pemphigus vulgaris which is an autoimmune dermatological disorder characterized by vesiculobullous lesions over the skin and mucosae may also give rise to cardiac disease. Global Longitudinal Strain (GLS) measurement may help to predict cardiac dysfunction in these patients.Method: In this prospective study, the global longitudinal strain was measured before and after steroid pulse therapy in pemphigus vulgaris patients using 2D speckle-tracking echocardiography. Moreover, blood pressure, pulse rate...
Source: Journal of Dermatological Treatment - Category: Dermatology Tags: J Dermatolog Treat Source Type: research
We report the case of a 13-year-old girl suffering from Crohn's disease (CD) and primary sclerosing cholangitis (PSC) who developed PV after application of a tissue expander for surgical removal of a giant congenital melanocytic nevus (GCMN). To the best of our knowledge, the case presented here is the first report of PV possibly associated with the application of a tissue expander and also the first report of coexistence of PV with either PSC or with PSC and CD in the same patient. Triggering or acute exacerbation of PV may be considered a possible side-effect of tissue expander application, especially in patients with a ...
Source: Acta Dermatovenerologica Alpina, Panonica, et Adriatica - Category: Dermatology Tags: Acta Dermatovenerol Alp Pannonica Adriat Source Type: research
ConclusionWhile corticosteroid in combination with either AZA or MMF is an effective therapeutic regimen for the treatment of pemphigus, MMF demonstrates a shorter time to achieve CR on therapy and has a significantly higher steroid-sparing effect.
Source: Dermatology and Therapy - Category: Dermatology Source Type: research
ConclusionsAdjunctive procedures that can facilitate a decrease in the cumulative dosages of corticosteroids and immunosuppressants have great value in the management of PV. CO2 laser vaporization is a safe procedure with minimal morbidity and no long-term side effects and should be considered as an adjunctive treatment option for management of recalcitrant lesions in patients with oral PV.
Source: Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology - Category: ENT & OMF Source Type: research
We present a case of a 50-year-old man with an unremarkable past medical history who smokes 20 cigarettes every day. For the last 3 months, he has complained of pain on the gums. Clinical examination showed multiple white plaques on the buccal gingiva of all 4 quadrants, with a verrucous appearance, some of which had a reddish area. Incisional biopsies were performed of the lesions from the gingiva with clinical diagnosis of proliferative multifocal leukoplakia as well as a microbiologic culture intake.
Source: Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics - Category: ENT & OMF Authors: Source Type: research
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