Blistering Skin Diseases by Lawrence S Chan download in iPad, ePub, pdf
During the non-bullous phase, the condition is often misdiagnosed as urticaria. Pemphigoid Pemphigoid is a general term for a group of related diseases characterized by blistering skin eruptions.
The exact, underlying reason why the immune system malfunctions in individuals with autoimmune blistering diseases is unknown. It is essential to start these patients on oral calcium and vitamin D supplements, as many of them will remain on prednisone for months. 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. There are several different categories of autoimmune blistering diseases including pemphigus, pemphigoid, IgA-mediated dermatoses and epidermolysis bullosa acquista. When the epidermis separates from the dermis a blister bulla may form.
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. Most blisters develop in response to irritation or injury of the skin. Plasmapheresis also is a well established form of treatment for severe cases of autoimmune blistering diseases.
It is characterized by blisters that rupture easily and cause painful erosions. In autoimmune blistering diseases, antibodies mistakenly attack proteins that are essential for the layers of skin to stick adhere together. Blisters heal leaving erosions, crust, and dyspigmentation. IgA Mediated Bullous Dermatoses IgA-mediated bullous dermatoses are disorders characterized by elevated levels within the body of a specialized protein known as immunoglobulin A IgA. Pemphigus is estimated to affect anywhere from.
These disorders are caused by a mutation to a gene that creates proteins necessary for the proper health and function of the skin. Blistering lesions eventually heal, sometimes with scarring. Rituximab has been used successfully in refractory cases. After the blisters heal, scars and small white bumps or cysts milia may remain. In linear IgA disease, autoantibodies react against structures found at the dermal-epidermal junction causing the epidermis and dermis to separate.
Linear IgA disease is characterized by blistering eruptions on the skin. The symptoms of dermatitis herpetiformis tend to come and go.
Antibodies react against foreign materials e. In most cases, the trunk and the upper arms or legs are affected. Pemphigus IgA, also known as intraepidermal neutrophilic IgA dermatosis, is characterized by the development of fluid-filled blisters on the skin. In these disorders, IgA has a particular tendency to accumulate in the skin. Generally, the less widespread an autoimmune blistering disease is, the easier it is to control.
Treatment Systemic corticosteroids provide the most rapid and effective control of disease. Diagnosis A diagnosis of an autoimmune blistering disease is suspected based upon identification of characteristic findings, a thorough clinical evaluation, and a detailed patient history. Paraneoplastic pemphigus is characterized by painful lesions affecting the mucous membranes, especially those found in the mouth and the lips. For more information, choose the specific disease name as your search term in the Rare Disease Database.
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