Table of ContentsView AllTable of ContentsSymptomsTypesCausesDiagnosisTreatmentCoping
Table of ContentsView All
View All
Table of Contents
Symptoms
Types
Causes
Diagnosis
Treatment
Coping
Pemphigusis a rare group of blisteringautoimmune diseasesthat affects both the skin and mucous membranes.
Why pemphigus occurs remains something of a mystery, but scientists have been able to establish that the immune system will suddenly target and attack proteins, calleddesmoglein, that help cells stick together.
Verywell / Jessica Olah
Before the advent of corticosteroids, the rate of mortality in people with pemphigus was around 75 percent within a year. It has drastically gone down since then.
Pemphigus vulgaris will generally affect mucosal membranes of the mouth first, causing multiple ulcers that can persist for weeks and months. In some cases, the oral lesions may be the only symptom. In others, blisters may develop on the skin, most predominately the upper chest, back, scalp, and face.
The blisters are generally ill-defined and easily ruptured. They will often consolidate into larger blisters and cause extensive peeling and oozing. The blisters are almost invariably painful, and, depending on their location, can be itchy or non-itchy.
If left untreated, pemphigus can gradually spread and involve greater amounts of tissue. This can lead to potentially life-threatening complications, including:
Death is most often the result ofsepticemia,infection, or pneumonia.
Pemphigus Vulgaris
Pemphigus vulgaris is the most common form of the disease. The sores most always originate in the mouth but may also affect other mucosal tissues such as the genitals.
Because the disease affects deeper tissues, the blisters can be extremely painful (although they tend not to itch). Only a small percentage of people will develop skin blisters as well.
Pemphigus vulgaris can sometimes occur as a feature of the neuromuscular autoimmune diseasemyasthenia gravis.
This photo contains content that some people may find graphic or disturbing.See PhotoReproduced with permission from © DermNet New Zealandwww.dermnetnz.org2023.
This photo contains content that some people may find graphic or disturbing.See Photo
This photo contains content that some people may find graphic or disturbing.
Reproduced with permission from © DermNet New Zealandwww.dermnetnz.org2023.
Pemphigus Foliaceus
Pemphigus foliaceus is a less severe form of the disease affecting the skin. It only involves superficial tissues on the dry top layer known as thestratum corneum. Because of this, the disease is far less painful but can often be extremely itchy.
Pemphigus foliaceus can sometimes affect people withpsoriasis, most often as a result ofultraviolet (UV) light therapyused to treat the autoimmune skin condition.
Other Types
There are other less common but potentially more serious forms of pemphigus, each of which has different underlying causes:
With pemphigus, the immune system will produce proteins called autoantibodies that are programmed to target desmoglein. Desmoglein is a protein that functions as an adhesion molecule, holding cells together to maintain tissue integrity.
With the exception of IgA pemphigus, the autoantibodies involved in pemphigus are immunoglobulin G (IgG). Certain types will target desmoglein 1 in superficial tissues (causing pemphigus foliaceus), while others will target desmoglein 3 in deeper tissues (causing pemphigus vulgaris).
Genetics
Genetics is believed to play a central role in the development of pemphigus. Certain genetic mutations are commonly seen in people with the disease, most of which involve the human leukocyte antigen (HLA) group of genes. HLA DR4 is the mutation most commonly seen in people with pemphigus.
Pemphigus disproportionately affected certain ethnic groups, including Ashkenazi Jews and people of Mediterranean origin. There are even certain subtypes that occur almost exclusively in Colombian and Tunisian populations.
Risk Factors
Despite the long list of known triggers, the majority of cases will be idiopathic (meaning of no known origin).
Pemphigus can mimic other diseases and usually requires a specialist, such as dermapathologist or oralpathologist, to render a definitive diagnosis. This typically involves a biopsy of the affected skin or mucosal tissues.
Under the microscope, the pathologist will look for fluid-filled lesions in the outer layer of skin (called intraepidermal vesicles). The vesicles provide clear evidence of acantholysis and help differentiate pemphigus from other blistering skin diseases.
A definitive diagnosis requires a technique known as direct immunofluorescence to identify anti-desmoglein autoantibodies. Under the microscope, the autoantibodies will appear as fluorescent deposits in the junctions between cells.
A blood test, referred to as an enzyme-linked immunosorbent assay (ELISA), can also be used to detect anti-desmoglein autoantibodies.
If the esophagus is affected, anendoscopymay be performed to view inside the windpipe and obtain tissue samples. X-ray and ultrasound are less helpful in rendering a diagnosis.
Differential Diagnosis
If the results are inconclusive, your healthcare provider will look for other possible causes of symptoms. Referred to as adifferential diagnosis, the investigation may include such diseases as:
If not treated immediately, pemphigus can be fatal, usually as the result of overwhelming opportunistic infections. Because of this, pemphigus may require hospitalization and involve many of the same interventions used in burn centers.
The mainstay of pemphigus treatment is oralcorticosteroids, usuallyprednisone. This typically requires extremely high doses, which can be dangerous for some, causing intestinal perforation and sepsis.
If oral corticosteroids cannot be used, other options may be considered, including:
Plasmapheresis, a technique similar to dialysis used to clean blood, may be considered if Rituxan cannot be used. Tetracyclineantibioticsmay also be prescribed to prevent secondary infections, while talcum powder can prevent bedsheets and clothing from sticking to the lesions. Many people get better with treatment, although it can sometimes take years to fully recover. Others will need to take medications permanently to prevent a recurrence.
Given that we know so little about what triggers pemphigus, it’s hard to suggest what you can do to avoid it if you have never had it before. With that being said, you may be able to prevent recurrence if you’ve had pemphigus in the past. Here are some self-help tips that can help:
A Word From Verywell
Pemphigus may be difficult to live with, especially if it affects your ability to eat, causes pains, or creates unsightly, oozing sores. Rather than isolating yourself, it helps to speak with others who understand what you are going through.
You can connect with online support communities on Facebook or contact the non-profitInternational Pemphigus and Pemphigoid Foundationto see if there is a support group in your area. If you are unable to cope, do not hesitate to ask your healthcare provider for a referral to a therapist or psychiatrist who can provide counseling and medications, if needed.
5 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.International Pemphigus and Pemphigold Foundation.Pemphigus.Arpita R, Monica A, Venkatesh N, Atul S, Varun M.Oral Pemphigus Vulgaris: Case Report. Ethiop J Health Sci. 2015;25(4):367-72.National Institute of Arthritis and Musculoskeletal and Skin Diseases.Pemphigus.Vodo D, Sarig O, Sprecher E.The Genetics of Pemphigus Vulgaris. Front Med (Lausanne). 2018;5:226. doi:10.3389/fmed.2018.00226Gregoriou S, Efthymiou O, Stefanaki C, Rigopoulos D.Management of pemphigus vulgaris: challenges and solutions. Clin Cosmet Investig Dermatol. 2015;8:521-7. doi:10.2147/CCID.S75908Additional ReadingAbréu-vélez AM, Reason IJ, Howard MS, Roselino AM.Endemic pemphigus foliaceus over a century: Part I.N Am J Med Sci. 2010;2(2):51-9.Kasperkiewicz M, Ellebrecht CT, Takahashi H, et al.Pemphigus. Nat Rev Dis Primers. 2017;3:17026. doi:10.1038/nrdp.2017.26Kridan, K.; Zelber-Sagi, S.; and Bergman, R.Mortality and cause of death in patients with pemphigus.Acta Derm Venereol.2017; 97:xx-xx.Kwan, H.; Kwon, I.; Chung, J. et al.Pemphigus foliaceus associated with psoriasis during the course of narrow-band UVB therapy: A simple coincidence?Ann Dermatol.2011;23(Suppl 3): S281-S284. doi:10.5021/ad.2011.23.S3.S281.Yamagami, J.Recent advances in the understanding and treatment of pemphigus and pemphigoid.F1000Res.2018;7:F1000 Faculty Rev-1360. doi:10.12688/f1000research.14474.1.
5 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.International Pemphigus and Pemphigold Foundation.Pemphigus.Arpita R, Monica A, Venkatesh N, Atul S, Varun M.Oral Pemphigus Vulgaris: Case Report. Ethiop J Health Sci. 2015;25(4):367-72.National Institute of Arthritis and Musculoskeletal and Skin Diseases.Pemphigus.Vodo D, Sarig O, Sprecher E.The Genetics of Pemphigus Vulgaris. Front Med (Lausanne). 2018;5:226. doi:10.3389/fmed.2018.00226Gregoriou S, Efthymiou O, Stefanaki C, Rigopoulos D.Management of pemphigus vulgaris: challenges and solutions. Clin Cosmet Investig Dermatol. 2015;8:521-7. doi:10.2147/CCID.S75908Additional ReadingAbréu-vélez AM, Reason IJ, Howard MS, Roselino AM.Endemic pemphigus foliaceus over a century: Part I.N Am J Med Sci. 2010;2(2):51-9.Kasperkiewicz M, Ellebrecht CT, Takahashi H, et al.Pemphigus. Nat Rev Dis Primers. 2017;3:17026. doi:10.1038/nrdp.2017.26Kridan, K.; Zelber-Sagi, S.; and Bergman, R.Mortality and cause of death in patients with pemphigus.Acta Derm Venereol.2017; 97:xx-xx.Kwan, H.; Kwon, I.; Chung, J. et al.Pemphigus foliaceus associated with psoriasis during the course of narrow-band UVB therapy: A simple coincidence?Ann Dermatol.2011;23(Suppl 3): S281-S284. doi:10.5021/ad.2011.23.S3.S281.Yamagami, J.Recent advances in the understanding and treatment of pemphigus and pemphigoid.F1000Res.2018;7:F1000 Faculty Rev-1360. doi:10.12688/f1000research.14474.1.
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
International Pemphigus and Pemphigold Foundation.Pemphigus.Arpita R, Monica A, Venkatesh N, Atul S, Varun M.Oral Pemphigus Vulgaris: Case Report. Ethiop J Health Sci. 2015;25(4):367-72.National Institute of Arthritis and Musculoskeletal and Skin Diseases.Pemphigus.Vodo D, Sarig O, Sprecher E.The Genetics of Pemphigus Vulgaris. Front Med (Lausanne). 2018;5:226. doi:10.3389/fmed.2018.00226Gregoriou S, Efthymiou O, Stefanaki C, Rigopoulos D.Management of pemphigus vulgaris: challenges and solutions. Clin Cosmet Investig Dermatol. 2015;8:521-7. doi:10.2147/CCID.S75908
International Pemphigus and Pemphigold Foundation.Pemphigus.
Arpita R, Monica A, Venkatesh N, Atul S, Varun M.Oral Pemphigus Vulgaris: Case Report. Ethiop J Health Sci. 2015;25(4):367-72.
National Institute of Arthritis and Musculoskeletal and Skin Diseases.Pemphigus.
Vodo D, Sarig O, Sprecher E.The Genetics of Pemphigus Vulgaris. Front Med (Lausanne). 2018;5:226. doi:10.3389/fmed.2018.00226
Gregoriou S, Efthymiou O, Stefanaki C, Rigopoulos D.Management of pemphigus vulgaris: challenges and solutions. Clin Cosmet Investig Dermatol. 2015;8:521-7. doi:10.2147/CCID.S75908
Abréu-vélez AM, Reason IJ, Howard MS, Roselino AM.Endemic pemphigus foliaceus over a century: Part I.N Am J Med Sci. 2010;2(2):51-9.Kasperkiewicz M, Ellebrecht CT, Takahashi H, et al.Pemphigus. Nat Rev Dis Primers. 2017;3:17026. doi:10.1038/nrdp.2017.26Kridan, K.; Zelber-Sagi, S.; and Bergman, R.Mortality and cause of death in patients with pemphigus.Acta Derm Venereol.2017; 97:xx-xx.Kwan, H.; Kwon, I.; Chung, J. et al.Pemphigus foliaceus associated with psoriasis during the course of narrow-band UVB therapy: A simple coincidence?Ann Dermatol.2011;23(Suppl 3): S281-S284. doi:10.5021/ad.2011.23.S3.S281.Yamagami, J.Recent advances in the understanding and treatment of pemphigus and pemphigoid.F1000Res.2018;7:F1000 Faculty Rev-1360. doi:10.12688/f1000research.14474.1.
Abréu-vélez AM, Reason IJ, Howard MS, Roselino AM.Endemic pemphigus foliaceus over a century: Part I.N Am J Med Sci. 2010;2(2):51-9.
Kasperkiewicz M, Ellebrecht CT, Takahashi H, et al.Pemphigus. Nat Rev Dis Primers. 2017;3:17026. doi:10.1038/nrdp.2017.26
Kridan, K.; Zelber-Sagi, S.; and Bergman, R.Mortality and cause of death in patients with pemphigus.Acta Derm Venereol.2017; 97:xx-xx.
Kwan, H.; Kwon, I.; Chung, J. et al.Pemphigus foliaceus associated with psoriasis during the course of narrow-band UVB therapy: A simple coincidence?Ann Dermatol.2011;23(Suppl 3): S281-S284. doi:10.5021/ad.2011.23.S3.S281.
Yamagami, J.Recent advances in the understanding and treatment of pemphigus and pemphigoid.F1000Res.2018;7:F1000 Faculty Rev-1360. doi:10.12688/f1000research.14474.1.
Meet Our Medical Expert Board
Share Feedback
Was this page helpful?Thanks for your feedback!What is your feedback?OtherHelpfulReport an ErrorSubmit
Was this page helpful?
Thanks for your feedback!
What is your feedback?OtherHelpfulReport an ErrorSubmit
What is your feedback?
