Pemphigus

Pemphigus

"Blistering skin"



Pemphigus involves blistering of the outer layer of the skin (epidermal) and mucous membrane. It's an autoimmune disorder where the immune system produces antibodies against specific protein in the skin and mucous membrane (I.e. the body's immune system mistakes the cells in the skin as foreign and attack them with antibodies). These antibodies produce a reaction that leads to a separation of epidermal cells.

Pemphigus is a very rare disease an occurs almost exclusively in middle-age or older people. About half of the cases of pemphigus begin with blisters in the mouth then follow with skin blistering.

There are three categories of pemphigus: vulgaris, foliaceus, and paraneoplastic pemphigus.

Pemphigus Vulgaris (PV), is the most frequently diagnosed form of pemphigus. Sores and blisters almost always start in the mouth. It doesn't affect any of the internal organs because the blisters can go as far down as the vocal cords. PV doesn't cause permanent scars unless there is infection associated with the lesions.

Pemphigus Foliaceus (PF), in PF blisters does not occur in the mouth. Crusted sores or blisters usually first appear on the face and scalp and later involve the chest and back. The blisters are superficial, often itchy, an disfiguring skin lesions can occur.

Paraneoplastic Pemphigus (PNP), PNP is the least common but the most serious form of pemphigus an occurs most often in someone who has been diagnosed with some sort of cancer. Painful sores skin lesions of the mouth, lips, and esophagus are almost always present. PNP can affect the lungs an cause hidden tumors.

Symptoms

Lesions or recurrent & relapsing blisters

Mouth ulcers

Skin ulcers that ooze, crust, or drain

Superficial skin peeling or detaching easily

Treatments

Usually involve two are more drugs, Severe cases are treated