Eventually, the chest and upper back may become involved. The scalp and face are usually affected first. Pemphigus foliaceus is characterized by multiple small blisters that quickly break apart to form itchy (pruritic), scaly, crusted lesions that affect the uppermost layer of the skin. In most people, pemphigus vulgaris first develops in the mouth, followed by blistering of the skin. It is characterized by blisters that rupture easily and cause painful erosions. Pemphigus vulgaris is the most common form of pemphigus. The two main types of pemphigus are pemphigus vulgaris and pemphigus foliaceus. The term pemphigus is a general term for a group of related autoimmune blistering diseases. Pemphigus, pemphigoid and IgA-mediated dermatoses can be further broken down into additional subtypes. ![]() There are several different categories of autoimmune blistering diseases including pemphigus, pemphigoid, IgA-mediated dermatoses and epidermolysis bullosa acquista. Associated symptoms depend on the location of blister formation, but can include gastrointestinal bleeding, difficulty swallowing or difficulty breathing. Mucous membranes line the esophagus and anus, the inside of the mouth, the nasal passageways, the genitals and the throat. In some autoimmune blistering diseases, blisters or lesions can also form on the mucous membranes, the thin, moist coverings of many of the body’s internal surfaces. In many cases, blisters can rupture, becoming open sores or wounds. In autoimmune blistering diseases, blisters form because the body creates antibodies that attack certain proteins required for the proper health and function of the skin. Most blisters develop in response to irritation or injury of the skin. ![]() When the epidermis separates from the dermis a blister (bulla) may form.Ī blister can be either tiny or large and consists of a fluid-filled bubble that forms underneath the surface of damaged or dead skin. Specialized proteins and structures are required for the dermis and epidermis to stick together. The two main structural layers of the skin are the epidermis – the outermost, protective layer of skin – and the dermis – an underlying layer that contains numerous specialized cells, tissues and structures. Five distinct layers make up the skin each layer is filled with specialized cells. In addition to serving as a protective barrier, the skin is involved in many additional functions of the body such as regulating internal body temperature. The skin is the largest organ of the body. For more information, choose the specific disease name as your search term in the Rare Disease Database. NORD has individual reports on many of the specific disorders classified as autoimmune blistering diseases. In recent years, new insight into the causes and development of these disorders has led to research into new therapies such as the development of drugs that target the specific antibodies which cause the symptoms of these diseases. In other cases, autoimmune blistering diseases if left untreated can eventually cause life-threatening complications. Although there is no cure for autoimmune blistering diseases, they can often be controlled with treatment. ![]() In some people, blistering lesions can cover a significant portion of the skin. The specific symptoms and severity of blistering diseases vary from one person to another, even among individuals with the same disorder. In autoimmune blistering diseases, antibodies erroneously attack proteins that are essential for the layers of skin to stick (adhere) together.
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