Pemphigus vulgaris is an autoimmune disorder that involves blistering and erosion of the skin and mucous membranes. It occurs almost exclusively in middle-aged or older people. The primary lesion of pemphigus vulgaris is a soft blister filled with clear fluid that appears on healthy or irritated skin. Many cases begin with blisters in the mouth, followed by skin blisters that may come and go. The blisters inside the mouth can make it hard for the person to eat. The rupture of blisters on the skin may be painful and limit the person's daily activities. Complications due to infections can be serious and the damaging nature of the blisters can cause loss of body fluids and protein. The exact cause of pemphigus vulgaris is unknown, but the blisters in pemphigus vulgaris are associated with the binding of antibodies to the skin cells. Treatment is aimed at reducing symptoms and preventing complications, and may include the use of corticosteroids, immunosuppressive drugs, and more recently immunotherapy. Pemphigus vulgaris may require long term treatment to keep it in remission.
Source: GARD Last updated on 05-01-20
80%-99% of people have these symptoms.
Click on a symptom to see definitions for associated terms.
|Atypical scarring of skin|
|Abnormal blistering of the skin|
|Adrenocorticotropic hormone deficiency|
|Cerebellar cortical atrophy|
|Recurrent cutaneous abscess formation|
|Recurrent opportunistic infections|
|Abnormal oral cavity morphology|
|Feeding difficulties in infancy|
Pemphigus vulgaris is an autoimmune disorder. In the case of pemphigus vulgaris, the immune system mistakenly produces antibodies against specific proteins in the skin and mucous membranes, known as desmogleins. These proteins form the glue that keeps skin cells attached and the skin intact. When desmogleins are attacked, skin cells separate from each other and fluid can collect between the layers of skin, forming blisters that do not heal. In some cases, these blisters can cover a large area of skin.
Although it is rare, some cases of pemphigus vulgaris are caused by certain medications. Among others, medications that may cause this condition include:
Emotional stress, thermal burns, ultraviolet rays, and infections have also been reported as triggers for pemphigus vulgaris.
While in many cases the exact cause of pemphigus vulgaris remains unknown, several potentially relevant factors have been identified.
Pemphigus is not contagious. It does not spread from person to person. Though there can be a genetic predisposition to develop pemphigus, there is no indication the disease is hereditary.
Last updated on 05-01-20
Recent advances in the treatment of pemphigus vulgaris have significantly improved the long term outlook for people with the disease. For most people, the disease can be controlled with prednisone (corticosteroids) in combination with other medications or therapies. However, both the use of steroids and the other medications or therapies can cause side effects that may sometimes be serious, including increasing the risk of developing a fatal infection. Pemphigus vulgaris and its treatments can be debilitating and may cause lost time at work, weight loss, loss of sleep, and emotional distress. Connecting with others facing the same concerns and challenges can help a person cope with the changes the disease causes in their life.
The International Pemphigus Foundation provides patient support services to help people with the disease cope with its effects.
Last updated on 05-01-20
The goal of treatment of pemphigus vulgaris is to promote and maintain remission, as well as avoid complications, such as infections. Medications and therapies are used to decrease blister formation and promote healing of blisters and reduce scarring. Once in remission, it is important to determine the minimal dose of medication necessary to control the disease process, since both long term use of steroids and other immunosuppressive drugs can increase the risk of other medical problems. Treatment is individualized, and takes into account the person's other health conditions.
The most common first line therapy for pemphigus vulgaris is:
Other immunosuppessives and immunotherapies may be used in addition to steroid therapy to reduce the risk of relapse or to reduce the dosage strength of the steroid being used. Again the choice of the additional drug or therapy (adjuvant therapy) must be balanced against the possible negative side effects.
Most common choices to be added to steroid treatment (first line adjuvant therapies) include:
Other choices may include:
Additional medications may be considered if a person does not achieve remission with a combination of steroids and one of the medications or therapies listed above.
Antibiotics, antivirals, and antifungals may be used to prevent or fight an infection. Wound care may include baths and wound dressings to help heal blisters and sores. Severe cases of pemphigus vulgaris may require hospital stays to receive proper wound care, as well as intravenous fluids and electrolytes if mouth ulcers are severe. Treatment may also involve pain medication or pain management therapies.
Last updated on 05-01-20
Do you have information about a disease, disorder, or syndrome? Want to suggest a symptom?
Please send suggestions to RareGuru!