General or Other | - Others | Pemphigus (Disease)
Pemphigus is a group of rare skin disorders that cause blisters of the skin or mucous membranes, such as in the mouth or on the genitals. Pemphigus is a rare group of blistering autoimmune diseases that affect the skin and mucous membranes. In pemphigus, autoantibodies form against desmoglein. Desmoglein forms the glue that attaches adjacent epidermal cells via attachment points called desmosomes.
Causes and Risk factors
When autoantibodies attack desmogleins, the cells become separated from each other and the epidermis becomes unglued, a phenomenon called acantholysis. This causes blisters that slough off and turn into sores. In some cases, these blisters can cover a significant area of the skin.
Diagnosis and Treatment
Usually a chronic condition, pemphigus is best controlled by early diagnosis and treatment, which may include medications or treatments similar to those used for severe burns. The less widespread your pemphigus is, the easier it may be to control. Pemphigus is recognized by a dermatologist from the appearance and distribution of the skin lesions. It is also commonly diagnosed by specialists practicing otolaryngology- head and neck surgery, periodontists, oral and maxillofacial surgeons (specialists qualified in both medicine and dentistry) and eye doctors as lesions can affect the eyes and mucous membrane of the oral cavity. If not treated, pemphigus can be fatal from an overwhelming infection of the sores.
The most common treatment is the administration of oral steroids, especially prednisone, and often in high doses. The side effects of cortico-steroids may require the use of so-called steroid-sparing or adjuvant drugs. The immuno-suppressant CellCept (mycophenolic acid) is among those being used. ...