Pemphigus is a condition that causes blisters inside the mouth, throat, nose, eyes, genitals, and on other parts of the skin. This is an autoimmune disease that causes the body to attack the skin and the mucous membranes. In this condition, the immune system attacks the desmogleins, which are the proteins that bind skin cells to each other. When these proteins are inhibited in their function, fluid can collect between the layers of the skin.
Pemphigus vulgaris affects the skin and the mucous membranes, and Pemphigus foliaceus only affects the skin. Each of the two types has different symptoms associated with it. In Pemphigus foliaceus, blisters and itchy and painful lesions on the skin are the most common symptoms. In Pemphigus vulgaris, blisters form on the mouth and other mucous membranes in the body. Some patients only experience blisters and skin lesions in the groin and under the arms.
Blisters often make their appearance in the mouth first in Pemphigus vulgaris. The skin can become so fragile that it will peel just by pressing on it with fingertips. Blisters are often painful and are present in the throat, the nose, the eyes, and the genitals.
In Pemphigus foliaceus, blisters often first appear on the scalp, the chest, or the upper back. They then spread out over the body. Blisters will often peel off in layers or in scales and be very itchy or very painful.
This condition is caused by an immune disorder that results in the immune system attacking healthy tissues in the body. When the proteins that allow skin layers to bind together are damaged by the disease, fluid becomes trapped between the layers, causing blisters and painful skin lesions. Pemphigus can be caused by tumours or certain medications. Researchers are not sure what causes Pemphigus to be expressed in some patients who carry the related genes that cause the condition.
The risk factors for Pemphigus are:
Pemphigus is usually diagnosed by a dermatologist. This condition usually requires that skin biopsies be done to help confirm that the patient’s symptoms are being caused by Pemphigus and not another skin condition with similar symptoms. Diagnosis usually requires a direct immunofluorescence test.
This condition requires treatment right away or it can eventually prove to be fatal in some patients. Many patients with severe Pemphigus require hospitalisation to bring their symptoms under control. The use of NSAIDs must be prevented in these patients as they can predispose the patient to excessive bleeding.
Treatment usually requires patients to be placed on immunosuppressant drugs and might also necessitate taking local steroid injections. Biologic drugs can also sometimes help break the disease cycle and intravenous gamma globulin might also be given in severe cases. Plasmapheresis has been shown to be effective in some patients as this process helps clean the blood.
Secondary skin infections are often treated with antibiotics, and talcum powder can be used to help prevent skin lesions from sticking to other surfaces and clothing. Many patients will require a lifetime of care for this condition to avoid flares that are life-threatening.
Prevention of this skin health condition is not possible since the condition is linked to genetics. Management is the best option for those who begin to show symptoms of the condition. Avoiding excessive exposure to sunlight and treating skin injuries right away can be key to preventing flares in patients who carry the gene for this immune disease. Managing stress and maintaining good oral health can also prevent flares in some patients.