Common Immunobullous Disorders Of The Skin
Immunobullous (blistering) diseases of the skin are autoimmune disorders. Any autoimmune condition involves the immune system “attacking” its own organs or tissues by producing autoantibodies (antibodies against the self). In the skin, there are proteins that attach epidermal cells to each other and proteins that attach the epidermis to the dermis. These proteins are the glue that keeps the skin intact. When these proteins are damaged by autoantibodies, the cells separate from each other and a blister appears. The two main forms of immunobullous disease of the skin are Pemphigus and Pemphigoid.
There are two major forms of Pemphigoid, Bullous Pemphigoid
Bullous Pemphigoid (BP) is a blistering disease of the skin caused by autoantibodies directed against skin proteins that connect the epidermis and the dermis. BP causes blisters, itching and sometimes pain. The majority of patients with BP experience remission within five years of initial diagnosis, but sometimes the disorder relapses. BP can often be treated with a combination of topical steroids and non-steroidal medications, but there are cases in which treatment with oral corticosteroids or immunosuppressants is required because of more severe or widespread blistering. BP blisters usually heal without scarring. Good wound care is important to promote healing and prevent infection and scarring.
Mucous Membrane Pemphigoid (MMP or Cicatricial pemphigoid) is a blistering disease that can arise on any mucous membrane surface including the nose, mouth, eyes, esophagus, larynx, urethra and anal mucosa. Scarring commonly occurs in affected areas and spontaneous improvements and remissions are rare. The affected organs dictate what treatment should be used. Systemic steroids are generally not adequate to control the progression of MMP. Dapsone is a drug that can be helpful in less severe cases of MMP. Azathioprine, mycophenolate mofetil and cyclophosphamide are immunosuppressant medications that are used in severe cases of MMP. It is important to treat this condition promptly to avoid scarring. Sometimes it is difficult to make the initial diagnosis of MMP; more than one biopsy may be needed to confirm the diagnosis and implement appropriate therapy.
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Pemphigus presents as blistering and raw sores inside the mouth or on the skin. In some people, both locations are affected. Pemphigus is derived from the Greek word
vulgarismost commonly occurs in people between the ages of 40-60, often of Jewish or Mediterranean descent. Pemphigus vulgarismost often affects the inside of the mouth.
foliaceusis characterized by crusty sores that often begin on the scalp, and may move to the chest, back,and face. It is not as painful as pemphigus and is often vulgaris, mis-diagnosedas dermatitis or eczema.
- In paraneoplastic pemphigus, painful sores appear on the mouth, lips, and the esophagus. This type of pemphigus is associated with underlying cancer. Paraneoplastic pemphigus usually results in fatal destruction of lung tissue (bronchiolitis obliterans).
- The diagnosis of any pemphigus variant relies on visual examination of skin lesions, skin biopsy which must include
- If not treated, pemphigus can be fatal, due to overwhelming systemic infection and fluid losses through the skin. Pemphigus can cause scarring in severe cases, or when secondary infections occur.
- The most common treatment is the administration of oral corticosteroids, usually prednisone, in conjunction with “
steroid sparing” agents or immunosuppressants. These drugs quiet the immune attack so that blistering stops and sores can heal.
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Our providers continue to maintain the highest levels of accreditation and progressive ongoing education to learn and understand the latest developments in medical dermatology. Each provider's education and bio can be viewed below.
Melanie Kuechle, MD
Dr. Kuechle is a native of Texas and a graduate of Baylor University College of Medicine. She completed her dermatology residency at...
Dr. Craig Birkby
Dr. Birkby is Board Certified by the American Board of Dermatology and has performed over 15,000 Mohs surgeries...