What is pemphigus?
Pemphigus is a group of skin disorders that cause pus-filled blisters or bumps. The lesions usually develop on the skin, but they also form on the mucous membranes (soft lining of the eyes, nose, mouth, throat, and genitals). The blisters are soft and easily break open to painful sores. Without treatment, they can spread to large areas of the body and the risk of infection is high.
Pemphigus is an autoimmune disease that occurs in healthy individuals. It is sometimes confused with other blistering autoimmune skin diseases such as bullous pemphigoid, lupus erythematosus, and helicopter-borne disease. Pemphigus is not contagious. It is a life-long condition that can be controlled with ongoing medical treatment.
Causes of pemphigus
Pemphigus is an autoimmune disease. This means that your immune system is attacking cells in your body – in this case, skin cells. In rare cases, certain drugs can trigger the disease. These include penicillin and some medications for rheumatoid arthritis. But for the most part, we don’t know what caused it. It is not contagious.
The immune system makes proteins called antibodies. Antibodies usually attack harmful foreign substances such as bacteria and viruses. Pemphigus Vulgaris occurs when the immune system mistakenly makes antibodies against proteins in healthy skin and mucous membranes.
Antibodies break the bonds between cells and collect fluid between the layers of the skin. This can lead to blisters and cuts on the skin. The exact cause of the immune system attack is unknown. Very rarely, some medications cause Pemphigus Vulgaris. These medications include:
- Penicillin, a chelating agent that removes certain substances from the blood.
- ACE inhibitors are a type of blood pressure medication.
Symptoms of pemphigus
Pemphigus causes blisters on the skin and mucous membranes. The blisters rupture easily and open sores appear, which dissolve and become infected.
The signs and symptoms of the two most common types of pemphigus are as follows:
- Pemphigus foliaceus: This type causes blisters on the chest, back, and shoulders. Blisters are more itchy than painful. Pemphigus foliaceus is not the cause of mouth blisters.
- Pemphigus Vulgaris: This type usually begins with blisters in the mouth and then on the skin or genital mucosa. The blisters are usually painful but do not itch. Blisters in the mouth or throat can make swallowing and ingestion difficult.
- Paraneoplastic pemphigus (PNP): PNP is associated with certain types of cancer. Blisters from inside the mouth and affect the lungs, which can lead to a fatal outcome. Sores to the mouth, lips, and esophagus are almost always present and various skin lesions occur. PNP affects the lungs. In some patients, the diagnosis prompts doctors to look for a hidden tumor. In some patients, the tumor is benign and the disease improves if the tumor is removed surgically.
- Mucous membrane pemphigoid (MMP): MMP affects the eyes, mouth, and throat. The clinical form of pemphigoid ocular psoriasis (OCP) can lead to blindness in respiratory compromise involving the deeper parts of the eyes and throat.
- Epidermolysis bullosa acquisita: This type has blisters on the skin with no involvement of the mucous surfaces. The blisters are usually smaller than pemphigoid.
- Bullous pemphigoid (BP): BP is limited to the skin where blisters appear mainly on the abdomen, groin, back, arms, and legs. The blisters can be itchy and painful.
Pemphigus is different from bullous pemphigoid, which is a vesicular skin condition that affects older people and can lead to death.
It defines the group of autoimmune intraepithelial bullae characterized by the loss of normal cellular synthesis (acantholysis) and the presence of pathogenic autoantibodies (mainly IgG) against epithelial synthesis molecules. It is divided into two main subtypes: Pemphigus Vulgaris and pemphigus foliaceus.
However, there are many disorders such as IgA pemphigus, IgE pemphigus, pemphigus herpetiformis, drug-induced pemphigus, major usher syndrome, and local pemphigus foliaceus; The dermatologist identified by the appearance and distribution of skin lesions.
Otolaryngology – It is also diagnosed by specialists in head and neck surgery, periodontists, oral and maxillofacial surgeons, and ophthalmologists as the lesions affect the eyes and the mucous membranes of the oral cavity. Internally, it is similar to the common diseases called lichen planus and mucous membrane pemphigoid.
An accurate diagnosis requires a skin or mucosa biopsy performed by a dermatologist or oral pathologist. A skin biopsy is taken from the border of the bleb, prepared for histopathology, and examined under a microscope. The pathologist looks for an intraepidermal vesicle caused by the separation of epidermal cells (acantholysis).
Thus, the superficial (upper) part of the epidermis slags, leaving the lower layer of cells on the “floor” of the vesicle. This lower layer of cells has a “serious appearance.”
The appearance of anti-desmoglein antibodies by direct immunofluorescence on skin biopsy also requires an accurate diagnosis. These antibodies appear as IgG deposits along the desmosomes between epidermal cells, reminiscent of wire mesh. Anti-desmoglein antibodies can also be detected in a blood sample using the ELISA technique.
Treatment for pemphigus
People with moderate to severe illness are hospitalized. In the hospital, raw skin surfaces require extraordinary care, similar to that given to those who suffer severe burns. Antibiotics may be needed to treat infections in broken blisters. The dressing protects the soluble and raw areas.
High doses of corticosteroids are essential for treatment. They can be taken orally (oral) or intravenously (intravenously) if the person is hospitalized. If the disease is controlled, the dose of corticosteroids is gradually reduced (damaged).
The drug also suppresses immunity (azathioprine, cyclophosphamide, methotrexate, mycophenolate mofetil, cyclosporine, or rituximab) if the person does not respond to treatment or the illness worsens when the dose is damaged. Immunosuppressive drugs may also be given to reduce the need for corticosteroids in people who need corticosteroids for a long time or in high doses.
Intravenous immune globulin is another treatment used for acute Pemphigus Vulgaris. Some respond well to discontinuation of drug treatment, while others may need to continue taking higher doses of the drug.
It can cause many serious problems. In all of them:
- Circulatory infections (sepsis)
- Can lead to an inability to eat, weight loss, and malnutrition
- Skin infections
- Some forms of the disease can lead to death if left untreated
The medicines you take for pemphigus can also cause serious side effects. They understand:
- Low bone density (osteoporosis)
- Arterial hypertension
At least 75% of people with this disease have no evidence of complete remission or disease after 10 years of treatment. Some people need to take medicine for the rest of their lives to prevent pemphigus symptoms from coming back.
Departments to consult for this condition
- Department of dermatology