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Pemphigus

Definition


Disease: Pemphigus Pemphigus
Category: Dermatological diseases
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Disease Definition:

The group of rare skin disorders that cause blisters on the skin or mucous membranes such as in the mouth or on the genitals is called pemphigus, which usually occurs in people in their middle or older age. This condition seems to be more likely to appear in people of Middle Eastern or Jewish descent, though it affects all races and cultures.


Pemphigus, which is often considered as a chronic condition, is best controlled when it is less widespread and by early diagnosis and treatment that might include drugs or treatments resembling those used for severe burns.

Work Group:


Prepared by: Scientific Section

Symptoms, Causes

Symptoms:

Pemphigus is characterized by blisters on the skin and mucous membranes that rupture easily, leaving open sores which might ooze and become infected. This condition has three primal forms and depending on its form signs and symptoms vary:


Pemphigus vulgaris:
It usually starts with blisters that are typically painful but not itchy in the mouth which then erupt on the skin, as well as on the mucous membranes of the genitals. This is most common type of the disorder.


Pemphigus foliaceus:
In this type, the blisters aren’t painful but appear to be itchy and crusty; often starting on the face and scalp then erupting on the chest and back. This form does not always affect the mucous membranes.  


Paraneoplastic pemphigus:
This type results in painful sores on the mouth, lips and in the esophagus, in addition to skin lesions; it might be associated with malignancy. This type of pemphigus could result in lesions in the lungs as well, causing progressive lung disease and breathing difficulty (dyspnea).

Causes:

Pemphigus is known to be an autoimmune disorder, but the exact thing that triggers it is still not known. The immune system usually attacks foreign invaders like harmful viruses and bacteria. But in the case of pemphigus, the immune system erroneously produces antibodies to attack healthy cells in the skin and mucous membranes, to be more specific, mostly proteins known as desmogleins. These proteins bind skin cells to each other. The antibodies binding to the skin result in acanthylosis, the separation of the cells of the top layer of skin (epidermis).


Occasionally, side effects of blood pressure medications or chelating agents can cause the development of pemphigus. When the medicine is stopped, this kind of pemphigus often goes away.


Paraneoplastic pemphigus results from the presence of additional antibodies to those seen in other kinds of the disorder. People who have cancer such as leukemia or lymphoma usually develop paraneoplastic pemphigus. The pemphigus blisters might catch the attention of doctors suspecting malignancy in cases in which cancer hasn’t been detected yet.


There isn’t any way of predicting who might be affected by pemphigus, and it is not a contagious disease, yet there is a higher risk of developing it when one:

 

  • Is of Mediterranean or Jewish descent.
  • Has myasthenia gravis, a chronic disorder characterized by muscle weakness and fatigue, or some other autoimmune condition, or has thymoma, a tumor of the thymus, which is an organ that produces an essential part of the immune system, the white blood cells called lymphocytes.
  • Is middle-aged or older.

Complications

Complications:

Complications of pemphigus might include infection of the skin and sepsis, which is when the bloodstream becomes infected. Systemic infection could be fatal.


Independent of the underlying cancer, the mortality rate is estimated to be 90% for paraneoplastic pemphigus; respiratory problems are included in the complications of this type.


Potential side effects of the medications used for the treatment of pemphigus, especially corticosteroids, could be included in other complications that might arise.

Treatments:

The goal of treatment for pemiphegus is reducing the signs and symptoms in addition to preventing complications. Less widespread pemphigus is easier to control, and treatment is most effective at early diagnosis. Depending on the severity of the disease, special treatment methods can be determined.


Mild pemphigus:
Getting outpatient treatment might be possible when the pemphigus is not too widespread. Treatment is usually based on corticosteroids, such as prednisone. However, using these corticosteroids for a long period of time or in high dosages might cause serious side effects, such as:

 

  • Osteoporosis
  • Weight gain
  • Increased chance of infection because they suppress the immune system
  • Diabetes
  • Redistribution of body fat, contributing to a round face (moon face)
  • Mood swings


Some of the medications that can be combined with corticosteroids include:


Immunosuppressants:
These drugs that help prevent the immune system from attacking healthy tissue have serious side effects, such as a high risk of getting infected. Some examples are azathioprine and methotrexate.


Antibiotics and antifungal medications:
Infections can be managed or even prevented with the use of these prescription medications.
Other medications might be prescribed as well, for instance dapsone, intravenous immunoglobulin and rituximab. In small clinical trials, these medications proved to be helpful in the treatment of pemphigus.


SEVERE PEMPHIGUS:
Widespread pemphigus might need hospitalization, and the treatment resembles the one for severe burns. The open sores make a person highly vulnerable to infection, and might be fatal when it spreads to the bloodstream. The affected person might additionally be administered medications along with the ones mentioned above, such as:


Intravenous feeding:
When mouth sores make it too painful to eat, intravenous feeding might be required.


Therapeutic plasmapheresis:
The fluid part of the blood known as plasma is removed from blood cells by a device known as a cell separator in this process. The goal is to get rid of the antidotes attacking the skin. After this, the plasma is replaced with intravenous fluids or donated plasma.


Anesthetic mouth lozenges:
These could help relieve the pain of mild to moderate mouth sores.


Fluids:
The patient may have to receive fluids intravenously, in addition to proteins and electrolytes, which are minerals like sodium, potassium and calcium that keep the balance of fluids in the body; because the patient might have lost bodily fluids due to the oozing of the sores.

Prognosis:

Not available

Expert's opinion

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