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Other Rare Skin Conditions

Pemphigus

Immunobullous diseases

Immunobullous diseases are uncommon skin conditions caused by autoantibodies directed against various skin proteins. These conditions primarily affect older adults and can cause significant pain in patients. In some cases, such as pemphigus, these disorders can even be fatal (see below). Due to these effects, immunobullous diseases impact the quality of life both physically and mentally. To confirm a diagnosis, usually a skin biopsy is needed.

 

Pemphigus

Pemphigus vulgaris is a rare autoimmune disease that causes blisters on the skin and mucous membranes, most commonly occurring in the mouth. This disease can affect anyone but most commonly appears between the ages of 30-60. Initially, blisters will appear in the mouth and on the genitals and then will develop on the skin’s surface a few weeks to months after. Pemphigus can cause extensive and life threatening blisters if the diagnosis is delayed, as well as bacterial and viral infections.

To confirm a diagnosis, a skin biopsy is taken. Once a diagnosis is made, treatment options include systemic (oral) corticosteroids and immunosuppressive drugs. Topical therapy can also be used in more mild cases and include topical steroid creams. Sometimes, more rapid treatment is needed and treatment starts before the diagnosis is confirmed.