Pemphigus

Common Name(s)

Pemphigus

Pemphigus is a group of rare autoimmune diseases that cause blistering of the skin and mucous membranes (mouth, nose, throat, eyes, and genitals).  This condition can occur at any age, but often strikes people in middle or older age. It is more common in people of Middle Eastern or Jewish descent than in other races and cultures. Pemphigus is a chronic disease which is best controlled by early diagnosis and treatment.  Treatment includes steroids to reduce inflammation, drugs that suppress the immune system response and antibiotics to treat associated infections.

There are three main types of pemphigus:

Pemphigus vulgaris Pemphigus foliaceus  Paraneoplastic pemphigus
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Pemphigus" for support, advocacy or research.

Logo
International Pemphigus and Pemphigoid Foundation

The IPPF provides direct access to innovative and effective support that promotes the very best health care, improves quality of life, stimulates community resources, advocates for favorable government policies, accelerates the pace of scientific discovery and is the world’s best source of information on pemphigus and pemphigoid. We are compassionate in understanding, tireless in service, and relentless in advocating. One person at a time, we make a difference, building a community of care and hope.

Last Updated: 8 Mar 2013

View Details

 

General Support Organizations

Not finding the support you need? Show General Support Organizations

 
 
Top

How do you compare to others with this condition?

Privately answer questions about your health. Let resources, you select, come to you.

Anonymously share and see how your answers compare with others with this condition while privately providing key pieces of information to medical researchers, disease advocacy groups, and others ONLY YOU select to help speed up cures and better alternatives.

 
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Pemphigus" for support, advocacy or research.

Logo
International Pemphigus and Pemphigoid Foundation

The IPPF provides direct access to innovative and effective support that promotes the very best health care, improves quality of life, stimulates community resources, advocates for favorable government policies, accelerates the pace of scientific discovery and is the world’s best source of information on pemphigus and pemphigoid. We are compassionate in understanding, tireless in service, and relentless in advocating. One person at a time, we make a difference, building a community of care and hope.

http://www.pemphigus.org/

Last Updated: 8 Mar 2013

View Details

 

General Support Organizations

Not finding the support you need? Show General Support Organizations

 
 
 
 
Top

Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Pemphigus" returned 651 free, full-text research articles on human participants. First 3 results:

Increased Frequency of T Follicular Helper Cells and Elevated Interleukin-27 Plasma Levels in Patients with Pemphigus.
 

Author(s): Tina Hennerici, Robert Pollmann, Thomas Schmidt, Maria Seipelt, Björn Tackenberg, Christian Möbs, Kamran Ghoreschi, Michael Hertl, Rüdiger Eming

Journal:

 

Pemphigus is an autoimmune disease in which IgG auto-antibodies (auto-ab) against the desmosomal cadherins desmoglein (Dsg) 3 and Dsg1 cause loss of epidermal keratinocyte adhesion. Aim of this study was to investigate cytokines derived from antigen-presenting cells (APC) and their ...

Last Updated: 13 Feb 2016

Go To URL
Multiplexed autoantigen microarrays identify HLA as a key driver of anti-desmoglein and -non-desmoglein reactivities in pemphigus.
 

Author(s): Thomas Sajda, Julian Hazelton, Milan Patel, Kristina Seiffert-Sinha, Lawrence Steinman, William Robinson, Brian B Haab, Animesh A Sinha

Journal: Proc. Natl. Acad. Sci. U.S.A.. 2016 Feb;113(7):1859-64.

 

Patients with pemphigus vulgaris (PV) harbor antibodies reactive against self-antigens expressed at the surface of keratinocytes, primarily desmoglein (Dsg) 3 and, to a lesser extent, Dsg1. Conventionally, only antibodies targeting these molecules have been thought to contribute to ...

Last Updated: 17 Feb 2016

Go To URL
Super-Resolution Microscopy Reveals Altered Desmosomal Protein Organization in Tissue from Patients with Pemphigus Vulgaris.
 

Author(s): Sara N Stahley, Maxine F Warren, Ron J Feldman, Robert A Swerlick, Alexa L Mattheyses, Andrew P Kowalczyk

Journal: J. Invest. Dermatol.. 2016 Jan;136(1):59-66.

 

Pemphigus vulgaris (PV) is an autoimmune epidermal blistering disease in which autoantibodies (IgG) are directed against the desmosomal cadherin desmoglein 3. To better understand how PV IgG alters desmosome morphology and function in vivo, biopsies from patients with PV were analyzed ...

Last Updated: 14 Jan 2016

Go To URL

Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Pemphigus" returned 46 free, full-text review articles on human participants. First 3 results:

Efficacy of rituximab for pemphigus: a systematic review and meta-analysis of different regimens.
 

Author(s): Hsiao-Han Wang, Che-Wei Liu, Yu-Chuan Li, Yu-Chen Huang

Journal: Acta Derm. Venereol.. 2015 Nov;95(8):928-32.

 

This meta-analysis examined the efficacy of different dosing regimens containing rituximab (RTX) in treating pemphigus. The analysis included 578 patients with pemphigus from 30 studies. Seventy-six percent of patients achieved complete remission (CR) after 1 cycle of RTX. Mean time ...

Last Updated: 4 Nov 2015

Go To URL
The dual nature of interleukin-10 in pemphigus vulgaris.
 

Author(s): Michael Jeffrey Cho, Christoph T Ellebrecht, Aimee S Payne

Journal: Cytokine. 2015 Jun;73(2):335-41.

 

The immunomodulatory cytokine interleukin-10 (IL-10) plays beneficial but also potentially detrimental roles in inflammation, infection, and autoimmunity. Recent studies suggest a regulatory role for IL-10-expressing B cells in the autoimmune blistering disease pemphigus vulgaris. ...

Last Updated: 18 May 2015

Go To URL
Non-classical forms of pemphigus: pemphigus herpetiformis, IgA pemphigus, paraneoplastic pemphigus and IgG/IgA pemphigus.
 

Author(s): Adriana Maria Porro, Livia de Vasconcelos Nasser Caetano, Laura de Sena Nogueira Maehara, Milvia Maria dos Santos Enokihara

Journal: An Bras Dermatol. ;89(1):96-106.

 

The pemphigus group comprises the autoimmune intraepidermal blistering diseases classically divided into two major types: pemphigus vulgaris and pemphigus foliaceous. Pemphigus herpetiformis, IgA pemphigus, paraneoplastic pemphigus and IgG/IgA pemphigus are rarer forms that present ...

Last Updated: 14 Mar 2014

Go To URL
 
 
Top

Symptoms, Diagnosis, and Treatment

There are currently no related results available in Genetics Home Reference.

There are currently no related results available in GeneReviews.

There are currently no related results available in Genetic Testing Registry.

 
 
Top

Clinical Trial Information This information is provided by ClinicalTrials.gov

Wound Dressings for Pemphigus and Pemphigoid
 

Status: Recruiting

Condition Summary: Pemphigus; Pemphigoid

 

Last Updated: 18 Feb 2015

Go to URL

Last Updated: 29 Apr 2011

Go to URL
Identification of Vulnerability Factors in the Course of Pemphigus Patients
 

Status: Recruiting

Condition Summary: Pemphigus; Dermatological Disease

 

Last Updated: 16 Aug 2016

Go to URL