Pemphiguscan occur on its own or manifest as a feature of certain autoimmune diseases or cancers.
Pemphigus may also be triggered by certain drugs.
The diagnosis typically involves abiopsyof the skin or mucous membrane.
Verywell / Jessica Olah
Treatment may include oral or injected steroids, immunosuppressant medications, intravenous antibodies, and biologic drugs.
It has drastically gone down since then.
In some cases, the oral lesions may be the only symptom.
Reproduced with permission from © DermNet New Zealandwww.dermnetnz.org2023.
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.
Types
There are several types of pemphigus that vary in their severity.
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.
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.
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.
Mouth sores do not occur.
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).
Genetics
Genetics is believed to play a central role in the development of pemphigus.
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
Pemphigus affects men and women equally.
This typically involves a biopsy of the affected skin or mucosal tissues.
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.
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.
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.
The problem can be further exacerbated by nonsteroidal anti-inflammatory drugs (NSAIDs) used to treat pain.
NSAIDs can cause gastrointestinal bleeding and may increase the risk of perforation.
Others will need to take medications permanently to prevent a recurrence.
Rather than isolating yourself, it helps to speak with others who understand what you are going through.
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