Pemphigus

Pemphigus is an autoimmune disease, meaning that cells that normally fight infection attack the body itself instead. It is a group of rare, chronic, progressive skin disorders that cause blisters and sores on the skin or mucous membranes, such as in the mouth, nose, throat, eyes or on the genitals.

The two main types are pemphigus vulgaris and pemphigus foliaceus.

Pemphigus vulgaris usually starts in the mouth. It can be painful.

Pemphigus foliaceus affects the skin and tends to be more itchy than painful. Regardless of type, the blisters are soft, limp, and break open easily. Anywhere the blisters form, they tend to break open quickly, leaving painful sores. In the throat, the sores can cause hoarseness. Mouth sores can make eating, drinking, and talking difficult.

Pemphigus is a rare group of blistering autoimmune diseases

When sores develop, they tend to heal slowly. Some may never heal. Pemphigus can occur at any age, but it’s seen who are between 40 and 60 years. Pemphigus is rare in children.

Pemphigus is not to be confused with bullous pemphigoid, another blistering skin condition. Usually a chronic condition, pemphigus is best controlled by early diagnosis and treatment. Treatment may include medications and therapies similar to those used for severe burns.

 

Pemphigus is characterized by blisters on the skin and mucous membranes. Pemphigus can develop slowly, causing blisters in the same area for years. But blisters can appear suddenly and spread. Widespread pemphigus can be life-threatening. It can turn an otherwise healthy person into one who is extremely sick, incredibly tired, and in pain. The following explains how pemphigus affects different areas of the body.

Pemphigus

Skin: On the skin, blisters usually begin in one area. They can develop on normal-looking skin or skin that looks inflamed. The blisters soon break open and ooze fluids. Then become sores partly covered with crust.

The sores are often painful, but rarely itchy. Some affected skin burns.

The sores tend to heal slowly, and some never heal. When a sore heals, may see a dark spot in its place. This is not a scar. Dark spots observed when the skin heals. The dark spots often fade, but this can take time.

Mouth and throat: Painful mouth sores are common who have pemphigus vulgaris. About 50% to 70% who have pemphigus vulgaris develop mouth sores before blisters appear on the skin.

Mouth sores begin as blisters, which quickly burst causing the painful sores.

These sores can be painful that affect eating solid food and drink (may use a straw). Talking can also be painful.

The blisters spread from mouth to lips and then skin.

Nails: Nail problems develop in who have severe pemphigus. An infection may develop in the skin around the nail. Sometimes observed nails slowly disappear.

Moist tissues: Painful sores can develop in the tissue lining the inside of the eyes and nose, genitals, anus, and other areas of the body. It can develop blisters and sores in rare cases.

Others: Pain, Fatigue, Weakness, Light sensitivity, Eye problems.

 

Pemphigus is an autoimmune disorder. It’s not contagious. In cases, it’s unknown what triggers the disease.

Normally, the immune system attacks foreign invaders. Like: harmful viruses and bacteria. But in pemphigus, the immune system mistakenly produces antibodies that attack healthy cells in the skin and mucous membranes.

Rarely, pemphigus develops as a side effect of medications, such as certain blood pressure drugs. This type of pemphigus usually disappears when the medicine is stopped.

Pemphigus cannot be cured, but with treatment, can control pemphigus. Treatment can reduce and sometimes clear the blisters and sores caused by all types of pemphigus. Treatment can also prevent pemphigus from worsening.

A treatment plan for pemphigus may include one or more of the following:

Corticosteroid: If have mild pemphigus, a corticosteroid may use & it can enough to control the disease. Some need stronger medicine like prednisone or methylprednisolone. These corticosteroids work throughout the body. A corticosteroid can clear the blisters and sores.

Immunosuppressant medication:  This medication quiets or suppresses the immune system. Either azathioprine or mycophenalate mofetil is often used to treat pemphigus. These can help to stop the body from creating new blisters.

Biologics: One biologic, rituximab may use. It appears to offer safe treatment.

Antibiotics, antivirals, and antifungals: If have an infection, this type of medicine will be part of treatment plan.

Wound care: Dermatologist may include baths and wound dressings in treatment plan. This can help heal blisters and sores.

Other medicines: These can be helpful for many reasons. For some need prednisone and the other medicines described above fail to control the pemphigus. Another medicine may work well. Switching to another medicine can also help prevent possible side effects.

When pemphigus is severe or medicine fails to work, a dermatologist may talk about one of the following treatment options:
Plasmapheresis: This treatment involves removing plasma from blood. Plasma contains the proteins that cause the immune system to attack the skin and the moist tissues lining mouth and other parts of body.

Extracorpeal photochemotherapy: This treatment begins with a blood draw. After white blood cells are removed from the drawn blood, the rest of the blood is returned to the body. The white blood cells are treated with a medicine called psoralen and then exposed to UVA light. This kills the diseased white blood cells. The treated blood is then returned to the body.

This treatment usually takes 2 days, but a patient can go home each day after treatment.

Hospital stays: To treat health problems that pemphigus can cause, some patients need to be hospitalized. While in the hospital, a patient may get an IV to replace lost fluids. Widespread sores can cause an enormous loss of fluids. An IV can also help patients get much-needed nutrition. Sores in the mouth or throat can make it too painful to eat.

In hospital, treatment can also be given to help get pemphigus under control.

If notice blisters that suddenly appear on skin, inside the mouth, or elsewhere, immediately make an appointment to see a dermatologist. Many skin diseases can cause blisters. But an accurate diagnosis is essential.

Take consultation if develop blisters inside the mouth or on the skin. If already have been diagnosed with pemphigus and are receiving treatment, see a doctor if develop:

  • New blisters or sores,
  • A rapid spread in the number of sores,
  • Fever, redness or swelling, which may indicate infection,
  • Chills,
  • Weakness or achy muscles or joints.
  • ABC Of Dermatology
  • Clinical Dermatology
  • Roxburgh’s common skin diseases
  • Andrew’s Diseases of the skin

Pemphigus

TUI - Tibot Urgency Index

Pemphigus is an autoimmune disease, meaning that cells that normally fight infection attack the body itself instead. It is a group of rare, chronic, progressive skin disorders that cause blisters and sores on the skin or mucous membranes, such as in the mouth, nose, throat, eyes or on the genitals.

The two main types are pemphigus vulgaris and pemphigus foliaceus.

Pemphigus vulgaris usually starts in the mouth. It can be painful.

Pemphigus foliaceus affects the skin and tends to be more itchy than painful. Regardless of type, the blisters are soft, limp, and break open easily. Anywhere the blisters form, they tend to break open quickly, leaving painful sores. In the throat, the sores can cause hoarseness. Mouth sores can make eating, drinking, and talking difficult.

Pemphigus is a rare group of blistering autoimmune diseases

When sores develop, they tend to heal slowly. Some may never heal. Pemphigus can occur at any age, but it’s seen who are between 40 and 60 years. Pemphigus is rare in children.

Pemphigus is not to be confused with bullous pemphigoid, another blistering skin condition. Usually a chronic condition, pemphigus is best controlled by early diagnosis and treatment. Treatment may include medications and therapies similar to those used for severe burns.

 

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Dr. Lora Smith

MBBS (Dhaka), DGO (DU) Ex SR. Gynaecologist & Obstetrician

09 606 111 222

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