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Autoimmune Bullous Disease

Autoimmune Bullous Diseases (ABD) are a group of rare skin conditions characterized by blistering and erosions on the skin and mucous membranes. These diseases arise when the immune system mistakenly attacks the structural proteins that holds the skin cells together. The exact trigger for this immune reaction is often unknown, but can sometimes be linked to genetics, environmental factors, and other autoimmune conditions.

Primary types of Autoimmune diseases may include:

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1. Pemphigus Vulgaris

Painful, soft blisters on the skin and mucous membranes, including the mouth, throat, nose, and genitals. In vulgaris, auto-antibodies targets proteins called desmoglein that hold skin cells together, leading to separation between cells in the top layer of the skin, causing blisters.

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2. Pemphigus Foliaceus

Fragile superficial blisters that are easy to break. In foliaceus, it follows the same attack mechanism as vulgaris, but less severe.​

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3. Bullous Pemphigoid [*Most Common*]

​Large, tense blisters that do not easily rupture. Often presents in people over 80 years of age, and commonly affects areas like the arms, legs, and abdomen. In bullous pemphigoid, auto-antibodies target proteins called BP180 or BP230 in the basement membrane zone, a layer that connects the epidermis to dermis.​​

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4. Mucous Membrane Pemphigoid (MMP)

MMP affects mucous membranes, such as those in the mouth, eyes, and genitals. In membrane pemphigoid, it follows the same attack mechanism as bullous pemphigoid, but less severe.

How can we help you?

Treatment aims to reduce blister formation, control symptoms, and prevent infection or complications. They may include:

  • Corticosteroids - these are primary treatment for ABD, and work to suppress the immune system.

  • Immunosuppressive Medications - these drugs may be prescribed to help reduce immune activity to allow for lower steroid dosages

  • Antibiotics or Antiviral Medications - these drugs may be prescribed to prevent or treat infections in open sores, especially in people on immunosuppressive therapy.

  • Topical Solutions/ Creams - Antiseptic solutions, steroid creams, or pain-relieving gels may help to manage local symptoms, particularly in the mouth.

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​Diagnosis for Autoimmune Bullous Diseases (ABDs): 

Diagnosing ABD requires thorough examination, as well as specialized test, to differentiate it from other blistering conditions. They may include:

  • Skin Biopsy - a small skin sample is taken from the affected area and examined to identify the level of skin separation and presence of immune cells.

  • Direct Immunofluorescence - detects antibodies in the skin by tagging them with fluorescent markers.

  • Blood Test - identification of specific circulating autoantibodies, helping to determine the exact type of ABD.

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