Pemphigus Vulgaris and Pemphigus Foliaceus: Differences in Epidemiology and Mortality.
The objective of this study was to compare PV and PF patients regarding ethnic variations and mortality rates. Mortality of PV and PF patients was compared with age- and gender-matched control subjects in the general population. The study cohort comprised 207 patients with PV and 30 with PF diagnosed during the period 2000 to 2015. The incidence rate of PV among Jews was 3.6-fold higher than among Arabs (p
Pemphigus foliaceus (PF) is one of the two main forms of pemphigus and is characterized by circulating IgG to the desmosomal cadherin desmoglein 1 (DSG1) and by subcorneal blistering of the skin. The pathomechanism of blister formation in PF is unknown. Previously we have shown that PF IgG induces aggregation of DSG1, plakoglobin (PG), and IgG outside of desmosomes, what in immunofluorescence of PF patient skin visualizes as a granular IgG deposition pattern with a limited number of coarse IgG aggregates between cells. Here we have investigated the fate of these aggregates in skin and found that these are cleared by endocy...
(University of Pennsylvania School of Medicine) Researchers from Penn compare a lymphoma-dose regimen of rituximab to a rheumatoid arthritis regimen for the treatment of pemphigus.
Mihika Dube, Brijesh Takkar, Dinesh P Asati, Sourabh JainIndian Journal of Dermatology 2019 64(6):499-500
This study aims to identify which disease severity index correlates best with the change in QOL. Fifty pemphigus patients completed QOL surveys with disease severity scored over two visits. QOL was assessed with the Autoimmune Bullous Disease Quality of Life (ABQOL), Dermatology Life Quality Index (DLQI), Skindex-29, and Short Form Survey 36 (SF-36). Disease severity was scored with the Pemphigus Disease Area Index (PDAI) and Autoimmune Bullous Skin Disorder Intensity Score (ABSIS). Correlations between the change in QOL scores and change in disease severity were analyzed using Spearman's coefficient (r). The change in PDA...
CONCLUSIONS: First-line treatment of pemphigus with rituximab results in a slightly greater cost compared with a standard CS regimen. PMID: 31657454 [PubMed - as supplied by publisher]
Interview with Aimee S. Payne, MD, PhD, author of Factors Associated With Complete Remission After Rituximab Therapy for Pemphigus
This cohort study evaluates the association between use of rituximab and remission in patients with pemphigus.
Mucocutaneous diseases affecting the oral cavity are predominantly immune mediated or inflammatory. Three of the most common and clinically significant conditions are reviewed in this article: lichen planus, pemphigus vulgaris, and mucous membrane pemphigoid. Because oral manifestations may be the first or only manifestation of mucocutaneous diseases, oral health professionals play a critical role in the early diagnosis and multidisciplinary management of these conditions.
Pemphigus foliaceus (PF) is an autoimmune blistering skin disease that occurs sporadically across the globe and is endemic in Brazil. Keratinocyte adhesion loss (acantholysis) is associated with high levels of anti-desmoglein 1 IgG autoantibodies, but the role of cell death is poorly understood in PF. Current evidence disqualifies apoptosis as the major cell death mechanism and no other process has yet been investigated. To approach the role of variation in genes responsible for cell death pathways in pemphigus susceptibility, we systematically investigated the frequencies of 1,167 polymorphisms from genes encoding product...