Pemphigus

Pemphigus Vulgaris – Rare Autoimmune Disorder 

Pemphigus vulgaris is a rare autoimmune disorder causing painful blisters on the skin and the mucous membranes. It does not spread to otherpeople on contact and does not seem to be inherited, but people’s genes are more at risk for pemphigus. Originally the cause of this disorder was not known and this disease was used in referring to any blistering disease of the skin and mucosa.

Researchers, in 1964, found that the blood of patients with pemphigus contained antibodies in the layers of skin which tend to separate forming these blisters. Pemphigus is a form of a group of autoimmune disorders which is collectively known as pemphigus and each kind of pemphigus is categorized based on the location where the blisters tend to form.

 Pemphigus vulgaris affects the mucous membranes that are found in the mouth, nose, throat, eyes, genitals and the lungs wherein this disease begins with blisters in the mouth and thereafter on the skin. The blisters at times also affect the membranes of the genitals. The disease could be serious with proper treatment being very essential and the use of corticosteroids to control the immune system is needed. If the treatment is neglected the condition could cause serious complication and could also end up being fatal.

Combination of Genetic/Environment Factors

Pemphigus vulgaris is an autoimmune condition which means that the immune system lacks the body’s defence against infection malfunctions and starts attacking healthy tissue. The immune system produces proteins known as antibodies which attack harmful alien substance like bacteria and virus and in case of pemphigus vulgaris, the immune system attacks cells that are found in deep layer of skin and the cells found in the mucous membrane causing blisters to develop in the affected tissue.

This could be due to the combination of genetic as well as environmental factors.Possible complication of this disease is when the blisters get infected where in some instances; the infection could spread into the blood stream and affect the entire body which could be very dangerous. Other complication could also comprise of side effects linked with long term use of corticosteroids and immunosuppressant.

Treatment & Side Effects

Physicians diagnose pemphigus through physical examination, a biopsy as well as blood test and the treatment for this disease comprise of steroids which helps in reducing inflammation, drugs which reduces the immune system response and antibiotics in treating the related infections. Presently no cure is available for this disease though the symptoms respond to high doses of steroid medication.

However, usage of high dose of steroids for a long period of time could cause serious side effects. Hence when the symptoms tends to clear, low doses should be used together with other medication which is known as steroid sparing therapy since it prevents some of the dangerous side effects related to long term steroid use. Most cases of pemphigus first tend to develop in older adults between the age group of 50 and 60 though this disease can affect people of all ages, including children. People of both genders are equally affected. It is more common in people of Jewish, Mediterranean and Indian origin.