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What is Pemphigus?
Pemphigus is a general term used to classify a group of rare autoimmune blistering skin disorders. These disorders occur when the body's immune system mistakenly attacks healthy tissue.
The exact cause of pemphigus is unknown, and the symptoms and severity associated with the various types of pemphigus vary. All forms of this disorder are characterized by blistering eruptions on the outer layer of the skin (epidermis). In pemphigus vulgaris, lesions also develop on the mucous membranes such as those lining the inside of the mouth. If left untreated, pemphigus will usually be fatal.
The two main types of pemphigus are pemphigus vulgaris and pemphigus foliaceus. Additional disorders may also be classified as pemphigus including paraneoplastic pemphigus and pemphigus IgA.
Pemphigus vulgaris (PV) is an autoimmune disease of the skin and mouth (mucous membrane). Auto-antibodies attack molecules that hold the skin cells together, causing them to separate and resulting in blisters. Pemphigus vulgaris is characterized by multiple lesions or blisters that do not heal, or that recur and spread to larger portions of the body about 80% of the cases first exhibit symptoms in the mouth, and the mouth may be the only site of lesions.
Typically, those with pemphigus vulgaris will have multiple ulcers that persist for weeks to months. Blistering may be accompanied by severe pain, itching, burning, and stinging and if extensive, blistering can lead to life-threatening fluid loss, infection, and disfigurement. Fatalities from pemphigus vulgaris are extremely unlikely in the United States; however, the timeliness of treatment is critical.
With treatment, lesions can heal normally without scarring. Most patients treated for pemphigus will enter partial or full remission within 2–5 years.
Who's At Risk?
Pemphigus vulgaris affects men and women equally. The average onset of symptoms is typically in the elderly, aged 50–60 years. Pemphigus vulgaris can occur in persons of any ethnicity, but the disorder tends to appear more often in persons of Mediterranean ancestry, Ashkenazi Jews, and some subtypes in people from Brazil and Colombia.
Signs and Symptoms
80% of the time, symptoms begin in the mouth, with soft (flaccid) blisters or erosions, and these symptoms are often mistaken for other conditions of the mouth. Two-thirds of those affected with pemphigus vulgaris eventually experience lesions on both the skin and in the mouth. If left untreated, the erosion/blisters gradually spread over an increasing surface area and can be complicated by severe infections, metabolic disturbances, or fluid loss.
When to Seek Medical Care
It is especially important to get an accurate confirming diagnosis early in the onset of this condition. If you suspect you might have pemphigus vulgaris, Call Us! We will talk to you and determine if you meet some of the key criteria to have the disease. We will also look to see if you display any of the signs or symptoms addressed above.
Early expert diagnosis is important, as pemphigus vulgaris – as with other autoimmune diseases – becomes more resistant to treatment if it is not promptly and appropriately controlled.
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