Autoimmune Blistering Diseases

Autoimmune Blistering Diseases

Autoimmune Blistering Diseases

Autoimmune blistering diseases refer to a variety of skin disorders where the body attacks healthy skin and tissue. This mistaken assault creates blister-like lesions that form on the skin, mucous, and membranes. Symptoms are patient-specific in their severity, ranging from minimal bumps to lesions that cover the entire body.

Our dermis is comprised of multiple layers that stick together through proteins, forming a protective barrier that performs a variety of functions, essential to human life. From regulating body temperature to protecting our blood vessels,  it’s crucial that the proteins making up this epidermal barrier remains healthy and intact. If afflicted with an autoimmune blistering disease, autoantibodies attack these proteins, prohibiting the epidermis from remaining healthy and intact. 

Different Types of Autoimmune Blistering Diseases and Their Risks

Pemphigus Vulgaris

The most common type of autoimmune blistering disease, pemphigus vulgaris, usually starts in the mouth as blisters, then moves to the skin. Blisters may erupt, creating painful sores and erosions on the surface of the skin. Blisters formed by pemphigus vulgaris aren’t typically itchy, however, when they burst and erode, could cause the patient to be more susceptible to infection. 

Pemphigus Foliaceus

These small, scaly blisters that quickly break away from the dermis usually don’t affect the mucous membrane or cause pain to the afflicted. However, the lesions caused by pemphigus foliaceus may cause the skin to become scaly and itchy and can affect the scalp, face, chest, and back. 

Paraneoplastic Pemphigus

Specific to individuals with cancer, paraneoplastic pemphigus is characterized by lesions that appear in the mouth and on the lips. This autoimmune blistering disease occurs on the body’s sensitive mucous membranes and may even move to the GI or respiratory tracts. When paraneoplastic pemphigus affects these areas, it can cause particularly life-threatening situations. 

Pemphigus IgA

Also called intraepidermal neutrophilic IgA dermatosis, this autoimmune blistering disease covers the skin with fluid-filled blisters. Upper arms and legs are most typically affected while the mucous membranes – like the eyes, nose, and mouth – remain blemish-free. 

Bullous Pemphigoid

A chronic skin condition that often affects the elderly population, firm blisters crop up on regular skin. These blisters usually appear reddish in color and are large in size. Sometimes they form near cuts or wounds or within skin folds, like the groin, armpit, and neck. Bullous pemphigoid lesions are extremely itchy but can heal somewhat quickly compared to the other autoimmune blistering lesions. 

Mucous Membrane Pemphigoid

This rare group of autoimmune blistering disorders is marked by blistering lesions that take up residence throughout the body’s mucous membranes. The nose, eyes, and mouth are often affected as well as the genitals and anus. Depending on the patient and site of lesions, symptoms may vary. Mucous membrane pemphigoid blisters typically heal on their own, however, in some cases, dermatological intervention may be necessary. 

Cicatricial Pemphigoid

Similar to mucous membrane pemphigoid. Cicatricial pemphigoid is also rare and may lead to scarring. When this blistering disorder affects the skin only or both the skin and mucous membranes, it’s referred to as cicatricial pemphigoid. When it only attacks the mucous membranes, it’s called mucous membrane pemphigoid. 

Bullous Dermatosis

One of the rarer autoimmune blistering diseases, bullous dermatosis occurs when the immune system attacks proteins within the subepidermal layer of skin. Cluster-like formations appear on the skin and cause intense itching. Any age can be affected by bullous dermatosis and a skin biopsy may help dermatologists determine how to proceed in treating it.   

Dermatitis Herpetiformis

This bumpy, itchy rash is particularly prevalent in those suffering from Celiac disease. The exact cause isn’t known, however, a gluten-free diet has been shown to mitigate random appearances of dermatitis herpetiformis. While dermatitis herpetiformis can quickly appear on the dermis, it usually does subside on its own. 

Pemphigoid Gestationis

Pregnant women, or those who have just given birth, are susceptible to pemphigoid gestationis. Red bumps or hives coalesce around the navel, arms, or legs. Itchiness occurs and the lesions turn into blisters. Pemphigoid Gestationis can resolve itself on its own and usually goes away within a few months of appearing. 

Treatment Options and Long Term Effects

For the specific types of lesions that don’t heal on their own, there are a variety of treatment options available. Dermatology deals with surgical, medical, and natural treatments for the epidermis. These specialist doctors have a wide array of experience dealing with blisters, lesions, scarring, and a variety of other skin diseases, making the dermatology office a good place to start when dealing with epidermal issues.  From holistic health remedies to modern medical intervention, here are some of the ways to treat these autoimmune blistering disorders. 

Blood Tests and Clinical Trials

In the case of autoimmune blistering disorders, blood tests ordered by a doctor who specializes in dermatology or endocrinology may help determine which of the skin diseases the patient is suffering from. Countless clinical trials have been performed to determine which treatment method works best for the various autoimmune blistering disorders, including the rare bullous dermatosis and mucous membrane pemphigoid. 

For example, rituximab, typically used for chemotherapy treatments, has been used in clinical trials successfully as a treatment for various autoimmune blistering disorders. A patient may even opt to participate in a clinical trial if certain treatments aren’t healing their blistering disorder. However, before going this route, consult with your dermatologist or primary care practitioner. 

Immunosuppressive Drugs and Corticosteroids

Certain drugs that suppress the immune system have shown to be effective when treating autoimmune blistering disorders, in particular bullous pemphigoid. A combination of dapsone, tetracycline, or other corticosteroids with immunosuppressant drugs can be administered intravenously under the supervision of an endocrinologist or other autoimmune or skincare specialist. 

Oral steroids, like prednisone, may cause severe side effects when taken alone, but be administered with immunosuppressive drugs. For example, prednisone and azathioprine, an immunosuppressive drug, may be mixed and given intravenously to combat blistering disorders. Corticosteroids and immunosuppressive drugs have proven themselves in many clinical trials as effective measures against many autoimmune blistering diseases. 

Immunoadsorption

Immunoglobulins are removed from the bloodstream as autoantibodies have been proven to be ultra-effective in perpetuating autoimmune blistering diseases. When intravenous immunoglobulin is pumped through the patient’s veins so the disposable immunoglobulin absorbs the autoantibodies, which have been attacking the person’s own proteins and creating blisters and lesions. 

Scarring from particularly large, painful lesions may occur but can be treated with the help of a dermatologist. Mentally, skin diseases are draining and usually cause the sufferer severe emotional distress. Not only are the blisters itchy, unsightly, and painful but they can leave lasting marks on one’s face, neck, hands, arms, or legs. 

Be Proactive and Take Action

Autoimmune blistering disorders are fortunately treatable and manageable, in most cases. For healthy individuals, it’s important to take action when noticing something new or abnormal on the skin. UCF Health’s team of skilled Orlando dermatologists, like Dr. Naveed Sami in Lake Nona,  are ready to help diagnose, treat, and manage autoimmune disorders, including blistering lesions. 

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