This page was last updated on February 14, 2023.
As news of coronavirus disease (COVID-19) changes frequently, one fact remains constant: The IPPF is dedicated to our community during this difficult time, and we are here to provide support and information. We are working hard to keep abreast of the situation and notify you about updates regarding the necessary precautions and recommendations to keep you safe during this pandemic. We will continue to update information on this page as it becomes available.
As always, we are listening to you during this time. When possible, we will try to answer general questions regarding the unique needs of pemphigus and pemphigoid patients. However, if you have specific questions or concerns about your condition, we recommend that you contact your physician.
IPPF Medical Advisory Council Statement: COVID-19 Update (Updated November 16, 2021)
Pemphigus and pemphigoid patients are recommended to receive an additional shot of the COVID-19 vaccine. Specific recommendations can vary based on whether or not you are currently taking immunosuppressive medications, described further below.
Why did the CDC recommend booster vaccines for immunosuppressed individuals as well as non-immunosuppressed individuals??
- People who are immunocompromised are more likely to transmit the virus that causes COVID-19 to household contacts.
- Studies suggest that fully vaccinated immunocompromised people accounted for nearly half of hospitalized breakthrough cases.
- Studies have shown that 40% of solid-organ transplant recipients who had no detectable antibody response to an initial mRNA vaccine series developed an antibody response to an additional dose.
- The SARS-CoV2 Delta variant is surging, and the number of people with COVID-19 is increasing in the United States, which puts immunosuppressed individuals at further risk.
Am I immunosuppressed?
- Pemphigus and pemphigoid patients who are not taking oral systemic therapies, and who have not received rituximab within the last 12 months, are generally not considered immunosuppressed and would not fall within the recent CDC guidance.
- You are considered immunosuppressed if you are receiving active treatment with:
- high-dose corticosteroids (i.e., ≥20mg prednisone or equivalent per day)
- rituximab within the last 12 months (rarely, patients who received rituximab more than 12 months ago may have prolonged B cell depletion and would be considered immunosuppressed).
- Topical steroids, IVIg, doxycycline, dapsone, and dupilumab are not considered immunosuppressive.
Should I ask for an antibody test to determine if I need a booster vaccine?
- Currently the CDC and FDA do not recommend blood tests to evaluate response to COVID-19 vaccines, except in the context of a research study.
Which booster vaccine should I get?
The FDA approved a “mix and match” approach, meaning that you can receive any of the available vaccines if the original vaccine you received is not available. The default choice is to receive the same vaccine you received for your original immunization.
(Recommendations from the CDC are updated frequently and as new information becomes available. For the most up-to-date recommendations from the US CDC, visit https://www.cdc.gov/coronavirus/2019-ncov/vaccines/recommendations/specific-groups.html)
- For Pfizer-BioNTech:
- Immunosuppressed: all individuals 12 and older who are at least 4 weeks out from their 2nd shot
- Booster for individuals 65 and older who are not immunosuppressed and are at least 6 months out from their last shot
- Booster for individuals 18 and older who are at least 6 months out from their last shot
- For Moderna:
- Immunosuppressed: all individuals 18 and older who are at least 4 weeks out from their 2nd shot, should receive a full-dose booster
- Half-dose booster for individuals 65 and older, who are not immunosuppressed and are at least 6 months out from their last shot
- Half-dose booster for individuals 18 and older who are at least 6 months out from their last shot
- For J&J/Janssen:
- All individuals 18 and older who are at least 2 months out from their last shot.
Do I need to delay or change any of my treatments for the vaccine?
- If you have been diagnosed with COVID-19 AND received monoclonal antibody therapy to treat infection, CDC recommends waiting at least 90 days to receive a COVID-19 vaccine or booster.
- For individuals who have received rituximab within the last 2 months or are anticipating additional rituximab infusions within the next 2 months, you may speak with your physician about delaying the booster vaccine until at least 3-4 months after rituximab infusion, or conversely delaying repeat rituximab infusion until at least 2 months after the booster vaccine. Your likelihood of responding to booster vaccination depends on the timing of vaccination in relation to rituximab infusion, along with the dose of rituximab received.
What other precautions should I follow?
- It is possible that the medications that prevented a response to initial vaccination will also prevent a response to booster vaccine. Hence, immunosuppressed individuals should follow general COVID-19 precautions, including:
- Wear a mask
- Stay 6 feet apart from individuals you don’t live with
- Avoid crowds and poorly ventilated indoor spaces without masking
- Close contacts should be encouraged to be vaccinated against COVID-19
Are there research studies in this field?
A clinical trial to study COVID-19 booster vaccine responses in pemphigus patients is currently enrolling at sites across the United States. You may wish to speak with a dermatologist at the study site to see if they are enrolling for the clinical trial. You must:
- Have a documented diagnosis of pemphigus (pemphigoid is not included)
- Have received 2 doses of either the Moderna or Pfizer COVID-19 vaccine, or one dose of the Janssen COVID-19 vaccine
- Must currently be taking mycophenolate (1000 mg daily or greater), methotrexate (7.5 mg weekly or greater), or have received rituximab within the last 12 months
- Have a negative or suboptimal response to COVID-19 vaccination based on a blood test performed at the time of screening
- Have relatively stable disease activity such that an increase in immunosuppressive medication is not anticipated at the time of screening
Full inclusion and exclusion criteria are listed at: https://clinicaltrials.gov/ct2/show/NCT05000216?term=pemphigus
IPPF Medical Advisory Council Statement: COVID-19 Vaccination (May 14, 2021)
Based on the current information that is available, the IPPF Medical Advisory Council recommends that all people that have been diagnosed with pemphigus or pemphigoid and are considered immunocompromised receive a COVID-19 vaccination in conjunction with discussing their vaccine and treatment strategies with their physician.
Vaccination Information Related to Autoimmune Bullous Diseases
The following information in this section originated from recommendations made by the European Reference Networks for Rare and Undiagnosed Skin Disorders (ERN-Skin) found here. This information has been reviewed and edited by the IPPF Medical Advisory Council to reflect the specific needs of pemphigus and pemphigoid patients.
Patients with autoimmune bullous diseases are at potential risk of having severe forms of viral infections, including COVID-19. This risk is particularly relevant in elderly patients and in patients taking oral steroid and/or immunosuppressive therapies such as methotrexate, mycophenolate mofetil, azathioprine, cyclophosphamide and rituximab.
The IPPF Medical Advisory Council, in accordance with the European reference center for autoimmune bullous diseases (MALIBUL), advises patients with autoimmune bullous diseases to be vaccinated against SARS-CoV-2.
The “mRNA vaccines” currently available are not live vaccines (i.e. they do not contain live attenuated virus or inactivated virus, but only the RNA for one small part of the virus). Thus, the benefit of vaccination is expected to outweigh any potential risk in patients with autoimmune bullous diseases, including those undergoing immunosuppressive treatment.
Concerning the effect of immunosuppressive treatments on the efficacy of vaccination, immunosuppression at the time of vaccination should be as low as possible in order to increase the chances of vaccine efficacy. However, if immunosuppressive treatment is underway, it should not be interrupted as this could lead to a relapse or flare-up of the disease.
In patients who are to be treated with rituximab, vaccination against SARS-Cov-2 should be completed 2 weeks prior to the start of rituximab treatment whenever possible. In other cases, it is best to wait 4-6 months after the last rituximab infusion since this is when the white blood cells (called B cells) start to rise.
Finally, it should be remembered that vaccination should not be accompanied by stopping other protective measures (wearing face masks, physically distancing, and hand-washing), especially in patients receiving oral steroid and/or immunosuppressive therapy, since it is currently unknown whether vaccinated individuals can transmit virus to non-vaccinated individuals.
Masks, Physical Distancing, and Hand Washing
The IPPF follows the recommendations for from the United States Centers for Disease Control and Prevention (CDC) in regard to masks and cloth face coverings, as well as the need for social distancing and hand washing. For the most up-to-date information, visit the CDC’s Considerations for Wearing a Mask page.
The IPPF maintains that the best way to protect yourself in the short-term is to stay at home to the extent possible; practice physical distancing; avoid touching your face; and wash your hands frequently. If you have to go out, wear a mask, use hand sanitizer, and wash your hands. Don’t go out if you are sick, unless you are seeking medical care.
Questions Related to Pemphigus and Pemphigoid
In response to some common questions we’ve received recently, the IPPF Medical Advisory Council has provided the following answers:
- Do COVID-19 vaccines cause bullous pemphigoid?
Bullous pemphigoid (BP) is the most common subepidermal autoimmune immunobullous disease. It characteristically affects the elderly, and its incidence has been rising. Given that there are hundreds of millions of vaccine doses administered each year, one would expect that by chance alone, there should be some individuals who develop BP following vaccination. However, to date, there have only been a relatively small number of reported cases of BP presenting after vaccination. Although the chance occurrence of BP following vaccination has led some to associate the two, carefully conducted studies have thus far not supported this conclusion. If you or a loved one has been diagnosed with BP after receiving a vaccination, it is most likely a chance occurrence.
- What precautions should patients with open P/P lesions do in light of COVID-19?
Direct spread through the skin is not a known source of transmission for COVID-19. Keep lesions clean and covered if around others.
- If not on any treatment, does simply having an autoimmune disease like P/P make you more susceptible to this coronavirus?
Pemphigus and pemphigoid patients who are not receiving immunosuppressive therapies are not known to be at greater risk for COVID-19.
- Does using topical treatments make you immunosuppressed?
Not usually. However, if the dosage is more than 20g of a class I steroid (clobetasol or betamethasone, etc.) some steroid systemic absorption occurs. It is possible this absorption can make a patient slightly immune-suppressed.
- How long does it generally take for the following medicines to get out of a person’s system and for their immune system to return to normal levels:
- Rituximab: The formal guidance is typically 1 year, although there is some variability in that response. We know from the published literature that many patients start to make new immune responses by 5-6 months after rituximab treatment.
- Corticosteroids (systemic prednisone or prednisolone): Within days to weeks, but these cannot be arbitrarily stopped and will need to have the dose weaned properly due to adrenal suppression.
- Class I Topical Steroids (Clobetasol/Betamethasone): These don’t affect the systemic immune system unless ~20g or more are applied daily. Even if high doses are used, these would “wash out” in days to weeks as above.
- Azathioprine/mycophenolate mofetil: These take 3 months to “wash out” of a person’s system.
- Cyclophosphamide: Cyclophosphamide should presumably take 3 months based on mycophenolate mofetil/azathioprine (MMF/AZA) data.
- Cyclosporine: Cyclosporine should presumably take 3 months based on MMF/AZA data.
- Dapsone: Dapsone doesn’t suppress the immune system in a way that would be expected to be problematic with COVID-19, and it “washes out” in a week or two.
- IVIg: IVIg doesn’t suppress the immune system
- Is IVIg therapy better than nothing?
Yes, there were randomized double-blind clinical trials of IVIg performed in Japan that showed that it was modestly effective at improving disease activity in bullous pemphigoid and also was beneficial in pemphigus. The main advantages of IVIg right now are that it is one of the only therapies for P/P that does not suppress the immune system. Additionally, it can be given by home infusion if your insurance approves that form of therapy.
- Does Rituxan put you more at risk of contracting a virus than being on high doses of prednisone?
Rituximab is expected to increase risk of viral infections and the severity of disease if infected. However, a randomized controlled trial published in Lancet (2017) showed that rituximab is better at controlling disease and resulted in a lower rate of infections compared to high-dose prednisone alone, so this issue would best be left to an individualized discussion with your doctor to determine the risk of disease versus the risk of treatment.
- Rituximab treatments have been postponed. What can be done in the meantime?
IVIg could be considered if the disease is significant, or topical steroids and other non-immunosuppressive measures if that is sufficient to control symptoms. However, severe disease should most likely still be treated, as the risk of hospitalization from severe disease could be worse than treating now to get disease symptoms under control and then self-isolating at home to avoid the risk of infection. Speak with your employer and doctor about flex hours or work-from-home options if immunosuppressive therapies are used.
- I work in a hospital where we are getting COVID-19 cases, and I’m on treatment for P/P. Should I stay home from work/take leave?
You may be at higher risk for severe manifestation of COVID-19. You should speak with your supervisors about opportunities to work at home or in isolation.
- Does being on long term, low-dose prednisone make COVID-19 symptoms come slower?
Published reports have suggested that prednisone at doses lower than 10 mg per day does not increase the risk of hospitalization from COVID-19 (Gianfresco et al, Annals of Rheumatic Diseases, 2020; 79:859). It is unknown but thought to be unlikely that prednisone would significantly delay symptoms of COVID-19 early in the disease course.
- I’ve been in remission, but now seem to be having a flare. Does taking medication put me at high risk for COVID-19?
Many oral and IV medications may increase the chance that you will have a more severe course of COVID-19. IVIg is likely an exception.
- When a vaccine for COVID-19 is available later in the year or next year, would rituximab’s effects on B cells affect the efficacy of the vaccine?
Rituximab is expected to decrease the efficacy of a COVID-19 vaccine if the vaccine is given in the first few months after rituximab.
- When should the vaccine be given in relation to the courses of rituximab? How many months before or after?
These issues will affect when the patients should get their next infusion. We do not know for certain, but it is reasonable to think that the ideal time to vaccinate for COVID-19 would be at least 4-6 months after rituximab and at least 1 month before rituximab.
- What process should an undiagnosed person follow if they suspect they have a bullous disease?
Call their local dermatology provider. Avoid the ER if possible. (NOTE: If you need assistance locating a dermatology provider in your local area who is experienced in dealing with pemphigus and pemphigoid, contact us.)
- Are there extra precautions that patients should observe as states begin to reopen?
Different states may be recommending different levels of precautions. Wear a mask or face covering in public; stay 6 feet away from others; wash your hands or use hand sanitizer if you are out in public spaces and avoid touching your face, nose, eyes, and mouth with unwashed hands; wash your hands immediately after returning to your home. Avoid mass transit if possible. Work places may offer flex hours or work-from-home options to help avoid “rush hour” when many commuters are using mass transit. If you cannot work from home or avoid mass transit – follow the guidelines above (face covering, physical distancing to the best extent possible, avoid touching your face with unwashed hands while commuting). If you develop fever, chills, cough, shortness of breath, sudden loss of taste or smell, headache, sore throat, muscle pain, call your primary care doctor for guidance and remember to tell them if you are on immunosuppressive treatments.
- Should patients (whether on or off treatment) go out into society and businesses as they begin to reopen, or should we stay home or request letters from doctors to continue to work from home until we know if there will be a big uptick in cases?
Patients who are off treatment have the same risk from infection as a non-pemphigus or pemphigoid patient. Patients who are on immunosuppressive therapy have greater risk of infection, as well as a more serious course of infection. Flex hours or work from home would be prudent if there is a resurgence of COVID-19 in your community. Speak with your employer and doctor about options.
- What precautions should be exercised if a member of the household has to work outside of the home?
Greater precautions are recommended if you are immunosuppressed and a member of the household works in a high-risk environment (for example, health care or a workplace that requires contact with multiple individuals.) Precautions the household member should consider include wearing a mask, frequent hand washing or use of hand sanitizer, avoid touching the face with unwashed hands while at work, washing their hands immediately after they return home, and surveillance for any of the symptoms of COVID-19 above.
The following links are intended to be used for reference only. Each link leads to a website that is not maintained by the IPPF; inclusion in this list does not equal endorsement by the IPPF. The information on the following websites may change at any time. This information is also not intended to be used as personalized medical advice. As always, discuss the specifics of your situation with your personal physician.
- British Journal of Dermatology: COVID-19 vaccine acceptance and hesitancy in patients with immunobullous diseases: a cross-sectional study of the International Pemphigus and Pemphigoid Foundation
- Pfizer-BioNTech: Global Information About Pfizer-BioNTech COVID-19 Vaccine
- World Health Organization: Coronavirus disease (COVID-19) Updates
- EURODIS: COVID-19 Resources
- EURORDIS: Open letter to policy makers: Recommendations to protect people living with a rare disease during the COVID-19 pandemic (PDF)
- European Reference Network ERN-Skin: Patients with rare skin diseases and COVID-19 virus
- Australian Ministry of Health
- New South Wales: Coronavirus FAQ
- Government of Western Australia Department of Health: COVID-19 Information
- RACGP: Coronavirus (COVID-19) Information for GPs
- Queensland Government: Novel coronavirus (COVID-19)
- South Australian Government: Coronavirus disease (COVID-19)
- Victoria State Government: Coronavirus disease (COVID-19)
- Association Pemphigus Pemphigoïde France: Informations sur le COVID-19
- Gouvernement: Informations Coronavirus
- Associazione Nazionale Pemfigo Pemfigoide Italy: Tutto su COVID-19
- Associazione Nazionale Pemfigo Pemfigoide Italy: INFEZIONE DA CORONAVIRUS
- Japanese Pemphigus and Pemphigoid Foundation: COVID-19 information
- Osaka City University: Policy on New Coronaviruses
- Ministry of Economy, Trade and Industry: Support Measures for Companies Concerning the Impacts of the Novel Coronavirus Disease
- Netwerk Nederland Pemphigus en Pemphigoid: CORONA en auto-immuun blaarziekten (AIBD) en aanverwanten
- British Association of Dermatologists: Dermatology Advice Regarding Self-Isolation and Immunosuppressed Patients: Adults, Paediatrics and Young People
- GOV.uk: Coronavirus (COVID-19): what you need to do
- GOV.uk: Public Health England
- NHS: COVID-19 Symptom Checker
- British Association of Dermatologists: Skin Support
- National Institute for Health and Care Excellence: Guidance
- U.S. Department of Health & Human Services: Joint Statement from HHS Public Health and Medical Experts on COVID-19 Booster Shots
- Centers for Disease Control and Prevention (CDC):
- American Autoimmune Related Diseases Association (AARDA): Should I Get the COVID-19 Vaccine? Newscast
- Biotechnology Innovation Organization (BIO): Covid Vaccine Facts
- Substance Abuse and Mental Health Services Administration (SAMHSA): Disaster Distress Helpline: 1-800-985-5990 and TTY 1-800-846-8517 -or- text TalkWithUs to 66746 (additional information on the SAMHSA website)
- National Domestic Violence Hotline: 1-800-799-7233 and TTY 1-800-787-3224 or chat on their website
- National Organization for Rare Disorders (NORD): Peter L. Saltonstall on Coronavirus Prevention and Risk for the Rare Community
- EveryLife Foundation: COVID-19 Resources
- USA.gov: Find your State Health Department
- Medicare.gov: Medicare and Coronavirus Information
- Centers for Medicare and Medicaid Services (CMS): Coronavirus (COVID-19) Partner Toolkit
- U.S. Department of Health and Human Services: COVID-19 Test to Treat Locator
- U.S. Food & Drug Administration (FDA):
- Global Genes: Coronavirus (COVID-19) Resources
- National Institutes of Health (NIH): Coronavirus (COVID-19) Information
- U.S. State Department: Information for Travelers
- American Public Health Association (AHPA): Get Ready Fact Sheet
- American Academy of Dermatology (AAD):
- National Health Council (NHC):
- Mental Health America: Mental Health and COVID-19
- Center for Chronic Illness: Programs and Services
- Emory University School of Medicine, Department of Dermatology: Patient Information
- The Organization for Safety, Asepsis, and Prevention (OSAP) and DentaQuest: Best Practices for Infection Control in Dental Clinics During the COVID-19 Pandemic
- Creaky Joints (arthritis and rheumatic disease): 6 Things That Don’t Need to Be ‘Perfect’ for Immunocompromised Patients to Benefit from the COVID-19 Vaccine
The IPPF provides peer support and information to all those affected by pemphigus and pemphigoid. Our community is strong and we are all in this together. If there’s anything we can do to support you, contact us at firstname.lastname@example.org.