Pemphigus Causes

Pemphigus Causes

 Pemphigus is a  autoimmune-related condition. Autoimmune disorders are caused when the body's natural defenses (e.g., antibodies) against "foreign" or invading organisms begin to attack healthy tissue for unknown reasons. Usually triggered by exposure to chemicals , dyes, trauma, surgery and exposure to infections. Carpet Shampoo is well known to trigger the blistering in Kawasaki disease.

Benign Familial Pemphigus (Hailey-Hailey disease) is inherited as an autosomal dominant trait. Human traits, including the classic genetic diseases, are the product of the interaction of two genes, one received from the father and one from the mother. In dominant disorders, a single copy of the disease gene (received from either the mother or father) will be expressed "dominating" the other normal gene and resulting in the appearance of the disease. The risk of transmitting the disorder from affected parent to offspring is 50 percent for each pregnancy regardless of the sex of the resulting child. More commonly seen in Jewish origin who intermarry specially of Persian origin.
 

 ( Pemphigus Foliaceus) is an autoimmune blistering disorder that may be triggered by a substance transmitted through the bite of blackflies.

Pemphigus may also occur following x-ray exposure or adverse reaction to drugs such as d-penicillamine or rifampin.

Affected Populations

Pemphigus affects males and females in equal numbers and is most common in middle-aged and elderly people. However, cases of children with Pemphigus have been reported. This disorder has been found in all ethnic groups and races, but is more common in people of Jewish or Mediterranean ancestry. Pemphigus occurs once in 100,000 people in the United States.

Related Disorders

Symptoms of the following disorders can be similar to those of Pemphigus. Comparisons may be useful for a differential diagnosis:

Bullous Pemphigoid is a chronic mild skin disorder that generally affects elderly people. It is characterized by large firm fluid-filled blisters (bullous pemphoid) that heal quickly and typically disappear in several months or years. However they may recur later. Early symptoms include redness on the skin followed within weeks by the appearance of blisters. The mucous membranes are rarely affected by Bullous Pemphigoid. (For more information on this disorder, see "Bullous Pemphigoid"

Darier Disease (Darier-White Disease or Keratosis Follicularis) is a progressive inherited skin disorder characterized by widespread firm elevated lesions on the skin and mucous membranes; abnormal changes of the finger and toe nails may also occur. Symptoms usually begin with a sensation of itching or burning on the skin, especially the scalp, forehead, face, neck, and back. Firm, elevated spots (papules) appear and typically become large and darkened; eventually these papules become scaly and crusty. As these spots enlarge, they may come together and form larger areas. The symptoms of Darier Disease tend to become more severe during periods of emotional stress or with exposure to sunlight. (For more information on this disorder, choose "Darier" as your search term on the Rare Disease Database.)