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Bullous Disease

There are several bullous diseases which can affect the skin and include bullous pemphigoid, dermatitis herpetiformis, epidermolysis bullosa acquisita foliaceous, linear IgA bullous disease, mucous membrane pemphigoid, pemphigus paraneoplastic pemphigus, pemphigus vegetans, and pemphigus vulgaris. A common symptom all these conditions share is the formation of blisters, resulting from the buildup of fluid between the cells of the skin on the uppermost layers (the epidermis and dermis). The disease types are classified depending on the severity of the blisters and where on the body they form. Many are considered autoimmune. The immune system is responsible for protecting and defending the body. When it malfunctions, it can attack the tissues of the body.

What Causes Autoimmune Diseases? While the specific cause of these particular diseases is still unknown, it is believed that environmental factors and genetic makeup may play a part in development. These conditions are not contagious, although some medications may result in a patient developing these specific disorders. These are most commonly found in elderly patients.

Bullous pemphigoid affects areas such as the arms, abdomen, inner thighs, groin, and areas of the face such as the eyes, nose, and mouth. These blisters will not easily break. They form between the dermis and epidermis layers. This condition frequently starts with hives or even a rash that may itch and can develop on the whole body or contained to just one specific area. This disease can last anywhere from months to many years.

Dermatitis herpetiformis affects the neck, elbows, buttocks, and knees with symmetrical erosions, bumps, and tiny blisters. Severe itching is a common symptom of this condition; it is also linked to gluten-sensitive bowel disease. Deposits of the IgA immunoglobulin are used to diagnose the condition. This condition is normally found in younger patients.

Epidermolysis bullosa acquista (EBA) is an autoimmune reaction to the patient's own collagen anchoring fibrils. There are also antibodies found in the blood. Symptoms of EBA include itching, hives, and blisters. The feet and hands are most commonly affected. When the skin lesions heal, they leave scars and tiny milia (cysts). It is most prevalent in middle age.

Linear IgA bullous disease is characterized by blisters that are often ring-shaped and can become very itchy. It is typically prevalent for middle-aged patients and is not associated with internal concerns.

Mucous membrane pemphigoid is known by the erosions and blisters that affect the mucous membranes of the eyes, nose, throat, mouth, and genitals. This condition can last for many years and typically leaves scars after healing. This condition often burns and itches.

Paraneoplastic pemphigus is less likely and more serious than other types of pemphigus because it may indicate an underlying tumor. A doctor may use specialized testing to identify this blistering autoimmune disease.

Pemphigus foliaceous is another type of pemphigus. It is itchier than other types of autoimmune blistering skin conditions. The chest and face are the most commonly afflicted areas. It can affect other areas of the body. The blisters easily rupture resulting in shallow erosions. Patients may experience moist, loose scales on their skin.

Pemphigus vegetans is a variant of pemphigus with thick erosions (sores) in the groin and under the arms.

Pemphigus vulgaris is the most frequently diagnosed form of pemphigus. Flaccid blisters, which are soft and limp, appear on mucous membranes and on skin that looks healthy, especially in areas of pressure. These blisters form within the epidermis, high in the skin, and may be painful. Skin may be "peeled" off by rubbing a finger on it (Nikolsky sign). When the blisters heal, patients are left with post inflammatory hyperpigmentation (dark spots). Pemphigus occurs frequently during middle age, and may last for years.

Diagnosis:
  • A detailed medical history and a physical examination may be required, noting the location and appearance of the associated skin lesions.
  • A skin biopsy. This is a procedure during which a section of the skin is removed to be viewed and examined closely underneath a medical microscope.
  • Direct immunofluorescence. This is a specific type of skin biopsy which is done by staining the skin to show different patterns and deposits of certain immune substances for detection and to provide an effective way to diagnose the exact type of bullous disease.
  • Indirect immunofluorescence. This is a blood test done to detect and measure the antibodies.

Treatment
  • Tetracycline may be beneficial in bullous pemphigoid.
  • Immunosuppressant drugs such as cyclophosphamide, azathioprine, or mycophenolate mofetil can be used with prednisone to help enhance the effects of the treatment
  • The doctor can prescribe sulfa drugs for conditions such as linear IgA bullous disease and dermatitis herpetiformis.
  • Prednisone or any oral corticosteroids. These are anti-inflammatory medications that can help in suppressing the immune system with large doses over time or lower doses to reduce unwanted side effects.

Bullous diseases can be controlled with the help of your dermatologist. This is why it is essential to seek medical care as soon as symptoms occur.

Useful Websites About Bullous Diseases


We put academic qualifications, many years of combined experience, and training in a variety of special interest areas to work for your healthy, radiant skin. Our providers – Drs. Schachter, Hanna, Curtis, Abdulla, Pollack, and Taradash – welcome new patients for treatment at the Dermatology on Bloor practice on Park Road in Toronto. Our team is here to serve your needs
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