It is an autoimmune disease, eventually fatal and characterized by causing skin disorders such as painful intraepidermal blisters and extensive erosions on the skin.
This condition occurs in middle-aged patients.
The two main types of pemphigus are pemphigus foliaceus and pemphigus vulgaris:
- Pemphigus vulgaris can be painful and usually begins in the mouth.
- Pemphigus foliaceus causes itching and affects the skin.
Causes of pemphigus vulgaris
The disease of the pemphigus is not contagious, it attacks the immune system, in most cases the cause is unknown.
The immune system under normal conditions attacks viruses and pathogenic bacteria. But in the case of pemphigus, the immune system mistakenly produces antibodies that attack healthy skin cells and mucous membranes.
The antibodies break the bonds between the cells forming a liquid that accumulates between the layers of the skin.
This induces the formation of blisters and subsequently erosions in the skin.
Pemphigus usually does not develop as an adverse effect of medications.
But in the case of penicillin (chelating agent) and some medications for blood pressure (ACE inhibitors), the appearance of pemphigus has been reported.
This type of pemphigus usually disappears when the medication is stopped.
Pemphigus presents characteristic symptoms, the formation of blisters that usually begin in the mouth, throat, nose, eyes and the mucous membranes of the genitals.
They can also occur in the upper part of the esophagus , which causes dysphagia and feeding difficulties.
These lesions are painful and these blisters break easily, leaving a bloody area with scabs and presence of exudates, or desquamations that tend to become infected.
In the event that the disease affects extensive body areas, the loss of fluids and electrolytes can be very significant.
Diagnosis of pemphigus vulgaris
The dermatologist begins the diagnosis with a physical examination of the chronic ulcerations, the diagnosis is confirmed with the biopsy of the skin lesions and the perilesional skin, called Biopsy with immunofluorescence tests.
When the symptomatology of the lesions points to the appearance of pemphigus vulgaris, it must be differentiated from other conditions that cause chronic oral ulcers and other bullous dermatoses such as:
- Drug eruptions.
- Erythema multiforme.
- Toxic epidermal necrolysis.
- Pemphigus foliáceo.
- Pemphigoid of the mucous membranes.
- Blister Penfigoid.
- Dermatitis herpetiforme.
- Bullous contact dermatitis.
The tests are performed through skin biopsies and serum antibody titers.
There are also two clinical findings, which show lack of epidermal cohesion, and which are specific to pemphigus vulgaris:
- Nikolsky’s sign: When a slight pressure or rubbing of the skin adjacent to an ampoule is exerted, the upper layers of the epidermis are displaced laterally.
- Asboe-Hansen sign: When the soft pressure is exerted on the intact ampoules, it is observed that the liquid containing the ampoule spreads outwards and below the adjacent skin.
Treatment of pemphigus vulgaris
Treatment is essential, and is aimed at reducing pain and symptoms and preventing complications. It usually involves the use of one or more medications to suppress the immune system.
Prescription medications may include corticosteroids (prednisone) and immunosuppressive drugs.
In general, a high initial dose is necessary to control the condition. It can cause serious complications, which can be fatal.
The use of these medications, have side effects, which include:
- A greater chance of infection.
- Diseases such as osteoporosis.
- Stomach ulcers
- Physiological problems such as loss of muscle mass and fluid retention.
It is suggested to take supplements such as vitamin D and calcium, a diet low in sugars and other medications are prescribed to treat these side effects.
Using the ampoules are controlled, the dose can be reduced to the minimum necessary to prevent the appearance of new blisters and to mitigate the side effects.
The use of a corticosteroid cream directly on the blisters is recommended.
To suppress the immune system and help keep the dose of corticosteroids low, additional medications are recommended.
As for example: azathioprine, cyclophosphamide, methotrexate , mycophenolate mofetil, rituximab, antibiotics, antiviral and antifungal.
The medical indications will depend on the degree of affection:
- Intravenous feeding: In case the canker sores are serious.
- Plasmapheresis : In very severe cases, this treatment is used, which is intended to eliminate antibodies, in this procedure the plasma or the fluid part of the blood is eliminated by means of a device and replaced by donated natural plasma.
- Wound treatment: Similar to that used for severe burns.