A cross-sectional study of the International Pemphigus and Pemphigoid Foundation

April 5, 2021
This past summer and early fall, we asked our patient community to participate in a survey regarding the effects that the ongoing COVID-19 pandemic have had on their lives. We’re happy to report that the results of that survey led to a publication in ​​​the Journal of The European Academy of Dermatology and Venereology this month: “COVID-19 Pandemic and Autoimmune Bullous Diseases: a Cross-Sectional Study of the International Pemphigus and Pemphigoid Foundation”.

We are especially grateful to the 372 pemphigus and pemphigoid patients who completed our USC / IPPF COVID-19 Impact Survey.

The objective of this study as outlined in the article was “to investigate the associations of outdoor activity restriction, income loss and treatment non-adherence with self-reported outcomes and to determine the satisfaction level with teledermatology platforms in patients with autoimmune bullous diseases (AIBDs) during the COVID-19 pandemic.” Access the article, including the study data and conclusions, here

The following is an excerpt from the article, “Breaking Bad: IgG4 in Autoimmunity” from Science Trends.

IgG4: friend or foe?


Our laboratory researches a rare and debilitating autoantibody-mediated disease called pemphigus vulgaris, in which the immune system generates antibodies against desmogleins, which are proteins responsible for holding skin cells together. The result is painful, sometimes deadly, blistering of the skin and mucous membranes, requiring powerful immune suppressants such as steroids, mycophenolate, and the anti-B cell agent rituximab for disease control. Because pemphigus vulgaris has a well-characterized target, we study it as a model autoimmune disease to better understand why and how the immune system makes mistakes, so that we can develop better ways of reversing these mistakes and hence, better treat disease.

Patients with pemphigus vulgaris are known to have autoantibodies of the IgA1, IgA2, IgG1, and IgG4 subclasses, with IgG4, in particular, being a marker of active disease.  As mentioned above, IgG4 is typically considered an anti-inflammatory antibody, induced during states of chronic immune stimulation to dampen disease.

Beekeepers and individuals undergoing allergic desensitization therapy develop IgG4 antibody responses that are thought to block symptoms caused by IgG1 or IgE reacting to the same stimuli, one of the reasons why people can outgrow an allergy from childhood. However, in pemphigus vulgaris, the antibody variable region is sufficient to disrupt skin cell adhesion and cause blisters, so trying to suppress these unruly variable regions with a law-abiding constant region can’t override this pathologic effect. Thus, although the immune system is trying all its usual tricks to stop the disease, the switch of B cells to the IgG4 subclass does not allow pemphigus vulgaris patients to outgrow or remit disease but instead becomes the defining immunologic feature of their disease.

Continue reading the full article on Science Trends.

This article was originally published in Science Trends on October 19, 2018.


Principia Biopharma Inc, a clinical-stage biopharmaceutical company dedicated to bringing transformative oral therapies to patients with significant unmet medical needs in immunology and oncology, today announced positive top-line data from the completed open-label Phase 2 trial of PRN1008 in patients with pemphigus (including both pemphigus vulgaris (PV) and pemphigus foliaceus (PF)) and the initiation of a Phase 3 trial of PRN1008 in pemphigus. The primary efficacy endpoint of the Phase 2 trial – Control of Disease Activity (CDA) within four weeks – was achieved by more than 50% of patients and PRN1008 was generally well tolerated. Based on the results of the Phase 2 trial, Principia has initiated the PEGASUS study, a global, randomized, double-blind, placebo-controlled, pivotal, Phase 3 clinical trial of PRN1008 in patients with moderate to severe pemphigus.

“Pemphigus is a debilitating disease with high unmet need. We are encouraged by the efficacy and safety results from the Phase 2 trial and are initiating the Phase 3 trial based on these results. This is a key milestone for Principia, and the next step toward bringing this novel oral therapy to patients in need,” said Martin Babler, Chief Executive Officer of Principia.

“PRN1008 has the potential to effectively treat patients’ disease and significantly reduce dependence on harmful corticosteroid (CS) doses, which have been the mainstay of treatment in this disease, and may usher in a new era of steroid-sparing, oral therapy for patients suffering from the disease,” stated Dr. Dedee Murrell, Professor and Head of the Department of Dermatology at The St. George Hospital Clinical School, University of New South Wales in Sydney, Australia and the lead principal investigator.

The full data from the Phase 2 trial will be submitted for presentation at a future scientific conference.

Read the full press release here. 

Syntimmune recently announced positive preliminary results from its phase 1b proof-of-concept trial of SYNT001 in pemphigus vulgaris and foliaceus patients. It’s exciting for the IPPF to share good news related to research and treatments. The full press release from Syntimmune can be found here. The following is an excerpt:

Syntimmune, Inc., a clinical-stage biotechnology company developing antibody therapeutics targeting FcRn, today announced positive preliminary results from its Phase 1b proof-of-concept trial of SYNT001 in patients with pemphigus vulgaris and pemphigus foliaceus. The data showed clinically meaningful benefit of SYNT001, with a favorable safety and tolerability profile similar to that observed in the Phase 1a study.

“There remains a clear unmet need for a safe and fast-acting treatment for patients with pemphigus, who face serious symptoms and complications associated with their disease,” said Donna Culton, M.D., Ph.D., an assistant professor at the University of North Carolina School of Medicine. Culton presented preliminary results of the Phase 1b study at the International Investigative Dermatology conference being held on May 16-19, 2018 in Orlando, FL. “These preliminary data demonstrate safety as well as a rapid reduction in PDAI scores and lowering of IgG levels with treatment of SYNT001, which support further studies of this drug as a potential new therapeutic option,” Culton said.

Read Syntimmune’s press release, including additional information, here. 

Stress can play a huge role in health conditions, especially autoimmune diseases.

Symptoms of stress include:

  • Poor or inadequate sleep (insomnia)
  • Anxiety
  • Depression
  • Panic attacks
  • Headaches
  • Poor concentration
  • Muscle aches
  • Skin inflammation (eczema)
  • Inflammation of the joints (arthritis)
  • High blood pressure (hypertension)
  • Stomach ulcers
  • Asthma attacks
  • Exacerbation of disease activity
  • Relapse of disease activity
  • and more

For those of you who have any one of the pemphigus/pemphigoid (P/P) related skin diseases, stress is the number one factor in flare-ups occurring. The mind-body connection is very strong and stress encourages the antibodies to act up and give you more blisters. Trying to stay even-minded is a big challenge. Easier said than done, but with practice, it can be accomplished! Any kind of exercise like yoga, swimming, or taking a brisk walk can be calming and fun at the same time! Meditation is also great tool for relaxing and can be done in the privacy of your home. If your mind wanders…just focus on your breath.

Deep breathing can help to calm down the feeling of anxiousness that stress can cause. You can inhale to the count of 10 (working up to 20), hold the breath for 10 and then exhale at the count of 10. Doing this with your eyes closed should help you to feel more relaxed.

Listening to music can be very relaxing. Dim the lights and lie down, breathe and just let yourself float away! YouTube has great choices if you do not already have your own easy listening library. Examples of music that you could search for are “yoga music,” “relaxing music for stress,” or “calming music for stress and sleep.” There many options available! Just type in the kind of music you want and YouTube has it.

Be creative in your de-stressing activities! Everyone finds activities to be relaxing, and it is important to find what best calms you. Some suggestions for de-stressing activities are:

  • Going for a walk with your dog
  • Going to the movies – especially to see a comedy (laughter can be the best medicine!)
  • Go for a hike
  • Adult coloring books
  • Photography
  • Fishing
  • Gardening
  • Cooking
  • Spending time with friends
  • Turning off all technology
  • Crafts
  • Go to the gym
  • Take art lessons
  • and more!

Anything you can do to take your mind off of the stress of having P/P will be helpful, and is your best tool in addition to what your physician prescribes.