Pemphigus: Treatment, Risks, and Genetic Factors

Pemphigus!

Hello, Doctor.
Previously, I mentioned my elder's condition, and you suspected it might be pemphigus.
My elder has now gone to Taichung Veterans General Hospital for examination and has been diagnosed with pemphigus.
I researched related information, and it seems that pemphigus is a serious illness that could even be life-threatening.
I would like to ask how pemphigus should be treated.
Additionally, I read online that the mortality rate can be as high as 80%.
Is it really that severe? Also, is this condition hereditary? Thank you, Doctor!

Mr. Zhang, 20~29 year old female. Ask Date: 2014/08/27

Dr. Gao Jiankai reply Internal Medicine

Dear Mr.
Zhang,
Thank you for your email.
Generally speaking, lupus erythematosus requires treatment with corticosteroids, and for patients who do not respond well to treatment, additional immunomodulatory medications may be necessary to control abnormal immune function.
For more details on this aspect, you may consult your dermatologist.
The hereditary risk of lupus erythematosus is low; not all children of patients with lupus erythematosus will necessarily develop the condition.
Other factors, including environmental influences, can also affect the likelihood of future flare-ups of lupus erythematosus.
Additionally, certain medications can trigger lupus erythematosus.
Therefore, similar to most autoimmune diseases, the exact causes of lupus erythematosus remain unclear.
Regarding the mortality rate of lupus erythematosus, the figure of 80% may be an overestimation.
Patients with this condition often require corticosteroids and immunomodulatory drugs, which can compromise their immune system.
Furthermore, some patients experience severe and difficult-to-control skin symptoms, placing them at a higher risk.
Consequently, treatment requires heightened vigilance and caution.
Sincerely,
Allergy, Immunology, and Rheumatology Department, Changhua Hospital, Ministry of Health and Welfare

Reply Date: 2014/08/27

More Info

Pemphigus is a rare autoimmune disorder characterized by the formation of blisters on the skin and mucous membranes. The condition arises when the immune system mistakenly attacks the proteins that help skin cells stick together, leading to the separation of these cells and the formation of blisters. Understanding pemphigus involves recognizing its types, treatment options, associated risks, and genetic factors.

Treatment of Pemphigus
The primary treatment for pemphigus involves the use of systemic corticosteroids, which help reduce inflammation and suppress the immune response. High doses may be required initially, followed by a gradual tapering as the condition stabilizes. In addition to corticosteroids, other immunosuppressive agents may be used, including:
1. Azathioprine: This medication helps to further suppress the immune system and is often used in conjunction with corticosteroids to reduce the required steroid dose.

2. Mycophenolate mofetil: Another immunosuppressant that can be effective in managing pemphigus.

3. Rituximab: A monoclonal antibody that targets CD20-positive B cells, which are involved in the autoimmune response. Rituximab has shown promise in treating pemphigus, particularly in cases resistant to conventional therapy.

4. Intravenous immunoglobulin (IVIG): This treatment can provide temporary relief by modulating the immune response.

In severe cases, hospitalization may be necessary for intensive treatment and monitoring, especially if there are widespread blisters or complications such as infections.

Risks and Mortality
While pemphigus can be a serious condition, the mortality rate is not as high as 80% in modern medical practice. Historically, before the advent of effective treatments, pemphigus vulgaris had a high mortality rate due to complications such as infections, dehydration, and electrolyte imbalances. However, with timely diagnosis and appropriate treatment, many patients can achieve remission and lead normal lives.

The risk of mortality can be significantly reduced with early intervention and proper management. It is essential for patients to work closely with their healthcare providers to monitor the disease and adjust treatment as necessary.

Genetic Factors
Pemphigus is not directly inherited in a simple Mendelian manner, but there is evidence suggesting a genetic predisposition. Certain genetic markers, particularly those related to the immune system, may increase the risk of developing autoimmune diseases, including pemphigus. Family history of autoimmune disorders can be a risk factor, but having a relative with pemphigus does not guarantee that other family members will develop the condition.

Conclusion
In summary, pemphigus is a serious autoimmune disorder that requires prompt and effective treatment to manage symptoms and reduce the risk of complications. While the condition can be life-threatening, advances in medical treatment have significantly improved outcomes for patients. It is crucial for individuals diagnosed with pemphigus to engage in regular follow-up care with their healthcare providers and to discuss any concerns regarding treatment, risks, and genetic factors. Early intervention and adherence to treatment plans can lead to better management of the disease and improved quality of life.

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