What Is Pemphigus Vulgaris?
Pemphigus vulgaris is the most common form of the autoimmune disorder pemphigus, which causes blisters in the mouth, then the skin, genitals and mucous membranes. The blisters rupture easily.
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Causes
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The immune system, which typically attacks viruses and germs, instead attacks healthy skin cells, specifically the desmoglein proteins. This causes the upper layers of flesh to separate. Some medications (like blood pressure meds) bring on pemphigus, as do some cancers, including lymphoma and leukemia.
Diagnosis
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Diagnosis is made by skin biopsy and immunofluorescence testing, which identifies the anti-desmoglein antibodies.
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Complications
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The complications of pemphigus vulgaris are similar to those of severe burns, because the open sores leave the patient vulnerable to infection. As a result, potentially fatal sepsis is a risk.
Drug Treatments
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The first level of treatment is typically corticosteroids (oral or intravenous) and immunosuppressants.
Fluid Treatments
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Intravenous fluids replace the fluids lost by the sores and restore the balance of electrolytes. Intravenous feeding also provides nutrition--eating is impossible due to the mouth sores.
Plasmapharesis
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The fluid plasma of the blood, which contains the desmoglein antibodies, is removed using a cell separator and replaced with donor plasma.
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