A Globally Available Internet-Based Patient Survey of Pemphigus Vulgaris: Epidemiology and Disease Characteristics

Gupta, Vibha; Kelbel, Theodore; Nguyen, Daniela; Melonakos, Katherine C.; Murrell, Dédée F.; Mullard, Andrew J.; Reed, Philip L.; Seiffert-Sinha, Kristina; Sinha, Animesh A.

doi:10.1016/j.det.2011.03.016

Cited by 33 publications

(40 citation statements)

References 65 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Most have shown a greater female:male ratio ranging from 1.17:1 to 2.25:1 (3,(10)(11)(12)(13)20). to our knowledge, this study is the largest dataset in PV published to date, and shows an increased female prevalence of 2.54:1. the reasons for the female preponderance in PV (and other autoimmune diseases) remain unclear.…”

Section: Discussionmentioning

confidence: 52%

“…to our knowledge, this study is the largest dataset in PV published to date, and shows an increased female prevalence of 2.54:1. the reasons for the female preponderance in PV (and other autoimmune diseases) remain unclear. one factor contributing to a female predominance in a disease registry such as this may relate to a potential underrepresentation of males participating in surveys, as has been previously documented (13), leading to a participation bias. it is known that women experience a more intense cellular and humoral immune response than men, perhaps rendering females more resistant to certain infections (due to a more potent response to infectious agents), but susceptible to higher rates of autoimmunity (20)(21)(22).…”

Section: Discussionmentioning

confidence: 93%

“…Patients with PV often experience a considerable delay in diagnosis after developing symptoms (13). the delay in diagnosis from the first occurrence of lesions was < 3 months for 120 patients (30.5%), 3-6 months in 131 patients (33.3%), 6-12 months in 89 patients (22.6%), and > 12 months in 53 patients (13.5%).…”

Section: Medical Carementioning

confidence: 96%

“…the rare nature of PV makes the collection of epidemiologic data of a substantial number of patients difficult and time consuming. to date, there have been few epidemiologic studies in PV, all conducted with limited data sets (3)(4)(5)(10)(11)(12), including an anonymous internet-based survey of 171 PV patients by our group (13).…”

mentioning

confidence: 99%

See 3 more Smart Citations

Development of a Disease Registry for Autoimmune Bullous Diseases: Initial Analysis of the Pemphigus Vulgaris Subset

Shah¹,

Seiffert-Sinha²,

Sirois³

et al. 2015

Acta Derm Venerol

View full text Add to dashboard Cite

Pemphigus vulgaris (PV) is a rare, potentially life threatening, autoimmune blistering skin disease. The International Pemphigus and Pemphigoid Foundation (IPPF) has recently developed a disease registry with the aim to enhance our understanding of autoimmune bullous diseases with the long-term goal of acquiring information to improve patient care. Patients were recruited to the IPPF disease registry through direct mail, e-mail, advertisements, and articles in the IPPF-quarterly, -website, -Facebook webpage, and IPPF Peer Health Coaches to complete a 38-question survey. We present here the initial analysis of detailed clinical information collected on 393 PV patients. We report previously unrecognized gender differences in terms of lesion location, autoimmune comorbidity, and delay in diagnosis. The IPPF disease registry serves as a useful resource and guide for future clinical investigation.

show abstract

Section: Discussionmentioning

confidence: 52%

Section: Discussionmentioning

confidence: 93%

Section: Medical Carementioning

confidence: 96%

mentioning

confidence: 99%

See 2 more Smart Citations

Development of a Disease Registry for Autoimmune Bullous Diseases: Initial Analysis of the Pemphigus Vulgaris Subset

Shah¹,

Seiffert-Sinha²,

Sirois³

et al. 2015

Acta Derm Venerol

View full text Add to dashboard Cite

show abstract

“…Infections caused by other herpesviruses such as CMV, VZV, EBV, or HHV-8 have also been described in association with PV patients [5,7,8,15,24,27,31,32,33,34]. CMV has been detected mostly in association with persistent lesions [20,29].…”

Section: Discussionmentioning

confidence: 99%

Laboratory Diagnosis of Herpesvirus Infections in Patients with Pemphigus Vulgaris Lesions

Oliveira-Batista¹,

Janini²,

Fernandes³

et al. 2013

Intervirology

View full text Add to dashboard Cite

Background/Aims: Pemphigus vulgaris (PV) is an autoimmune disorder that has an etiology impacted by genetic and exogenous factors. Viral infections, in particular herpesvirus infections, have been identified as possible PV triggers which in addition cause serious complications in these patients. This study investigates the frequency of herpesvirus infections in patients with PV lesions. Methods: Polymerase chain reaction and DNA sequence analyses were used to determine the presence of herpes simplex virus (HSV)-1/2, varicella-zoster virus (VZV), Epstein-Barr virus (EBV), cytomegalovirus (CMV), and human herpesvirus types 6, 7, and 8 (HHV-6, HHV-7, and HHV-8) DNA in 105 mucosal and/or skin swabs harvested from 23 patients presenting with PV lesions. Results: Thirty-six samples collected from 17 patients were positive for at least 1 virus; 3 were positive for HSV-1, 2 for HSV-2, 2 for CMV, and 7 each for HHV-6 and HHV-7. Coinfections were observed in 15 samples. Infections with VZV, EBV, HHV-6A, and HHV-8 were not detected. Herpesvirus infections were detected in 21% (13/62) of reactivated lesions, 54.5% (18/33) in persistent lesions, 40% (2/5) in exacerbated lesions, and 60% (3/5) in lesions in remission. Conclusion: PV lesions which do not show improvement following administration of adequate immunosuppressive therapy should be screened for the presence of herpesvirus infections.

show abstract

The genetic basis of pemphigus vulgaris

Vodo

Sprecher

2023

JEADV Clinical Practice

View full text Add to dashboard Cite

The severe autoimmune blistering disease pemphigus vulgaris (PV) is most probably mainly due to autoantibodies directed against desmosomal components of the epithelium, disrupting cell‐cell adhesion. The precise mechanisms underlying the development of the disease are still unknown, and immunosuppressive medications such as corticosteroids, which are linked to potentially serious adverse effects, are still the mainstay of treatment. Ethnic susceptibility and familial incidence hint to a genetic component to the pathophysiology of PV, which, if identified, could further improve our understanding of PV pathogenesis and lead to the discovery of new therapeutic targets for this potentially fatal condition. In this article, we review the evidence supporting a genetic component in PV and discuss the clinical and therapeutic implications of these discoveries.

show abstract

A Globally Available Internet-Based Patient Survey of Pemphigus Vulgaris: Epidemiology and Disease Characteristics

Cited by 33 publications

References 65 publications

Development of a Disease Registry for Autoimmune Bullous Diseases: Initial Analysis of the Pemphigus Vulgaris Subset

Development of a Disease Registry for Autoimmune Bullous Diseases: Initial Analysis of the Pemphigus Vulgaris Subset

Laboratory Diagnosis of Herpesvirus Infections in Patients with Pemphigus Vulgaris Lesions

The genetic basis of pemphigus vulgaris

Contact Info

Product

Resources

About