What is Pemphigus Vulgaris?

An autoimmune disorder that is characterized by sores or blisters on the skin and mucus membranes is called pemphigus vulgaris. Its cause is unknown but is sometimes from medications. ACE inhibitors and penicillamine are some medications that may cause this condition. It is an uncommon disorder and is predominantly found in middle-aged or older persons.

Symptoms

Half of all people with this condition have blisters and sores in the mouth first and then get them on the skin. The sores can go and then come back again. Sores can be in mouth, on trunk, scalp, or other locations and be described as crusting, oozing, draining, peeling, or easily detached.

Diagnosis

Skin biopsies, where a small piece of tissue is removed and tested, or a positive test for Nikolsky's sign (where skin comes off easily if rubbed) are ways to diagnose this condition.

Treatment

Treatment can include antifungals and antibiotics, IV fluids and electrolytes, IV feedings, numbing mouth lozenges, and pain medications. Other body-wide treatments include corticosteroids, gold containing medications, anti-inflammatories, and immunosuppressive therapy. Another treatment option is plasmapheresis.

Plasmapheresis

This is where antibody containing plasma is taken out of the body. In its place, donated plasma or IV fluids are put back in. This lessens the volume of antibodies in the blood and when used with systemic medications can help those with this condition.

This is a life-threatening condition without treatment. It can lead to death. Possible complications can be sepsis, severe dehydration, secondary skin infections, and possible side effects from the medications being used to treat the condition.

Source: A.D.A.M.