What is Pemphigus Vulgaris?
Pemphigus Vulgaris (PV) is an autoimmune disease of the skin and mouth (mucous membrane). Auto antibodies attack molecules that essentially hold the skin cells together, causing them to separate and resulting in blisters.
What are the features?
Pemphigus Vulgaris is characterized by multiple recurrent blisters that do not heal and persist for weeks to months. As many as 80% of the cases first exhibit symptoms in the mouth, and the mouth is sometimes the only site of lesions.
Typically, those with Pemphigus Vulgaris will have multiple ulcers accompanied by severe pain, itching, burning, and stinging, which persist for weeks to months. If extensive, blistering can lead to life-threatening fluid loss, infection, and disfigurement.
With treatment most patients treated for pemphigus will enter a partial or full remission within 2–5 years.
Who’s at risk?
Pemphigus Vulgaris affects men and women approximately equally. It is an ultra-orphan disease (meaning it is very rare).The average onset of symptoms is in those aged 50–60 years. The disorder tends to appear more often in persons of Mediterranean ancestry, Ashkenazi Jews, and some subtypes in people from Brazil and Colombia.
What are the signs and symptoms?
Eighty percent 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.
What is the treatment?
- Keep wounds clean.
- If bandaging is necessary, use bandages that provide moisture and protection.
- Avoid spicy foods (eg, garlic and onions) and nuts, which may help relieve symptoms.
- Antibiotic ointment or cream can help treat infection
- Oral rinses and ointments can be used to reduce pain in the mouth.
- A confirming diagnosis for pemphigus vulgaris is accomplished through a biopsy of the skin or the inner lining of the mouth (oral mucosa). Treatment begins with a period of intense therapy with corticosteroids (eg, prednisone) in an effort to gain control and suppress disease activity until no new lesions appear.
- After all lesions heal, medications can gradually be tapered, aiming for the lowest dose that prevents new “flares” of lesions from appearing.
- IVIG treatment, plasmapheresis, or biologic agents are second line therapy.
What would be the cost of the treatment?
A single consultation with Dr. Rinky Kapoor would cost about INR 1000/-. The cost for treating a case of Pemphigus depends upon the type of treatment chosen. First line therapy including corticosteroids costs much lesser than the second line therapy, such as Plasmapheresis. Plasmapheresis includes the process for separating the blood, or plasma, from the cells. Treatment with Intravenous Immunoglobulin (IVIG) also costs more than corticosteroid treatment.
Pemphigus if not treated, can be fatal. Most of the times, death may occur within first few years of disease. However, if the patient survives for 5 years, prognosis is good. Generally children affected with Pemphigus respond better to treatment.
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The Esthetic Clinic is a world class center dedicated to skin care and plastic surgery of the entire body – and specializes in housing an experienced Dermatologist, Dr. Rinky Kapoor, who can provide world class Skin Treatments, for skin diseases and make your skin look beautiful. So if you want to go ahead and get your hair, skin, and nails treated, The Esthetic Clinic is the best place for you.
About Dr. Rinky Kapoor
Dr. Rinky Kapoor, Co-founder of The Esthetic Clinic, is one of the best dermatologists in the world & currently practices in Mumbai, India. Dr. Rinky Kapoor is a Consultant Cosmetic Dermatologist, Cosmetologist & Trichologist at S L Raheja Fortis Hospital, Mumbai, India. Dr. Kapoor is trained at the National Skin Centre, Singapore & at Stanford University, USA. A celebrity skin doctor, Dr. Rinky Kapoor has won many honors such as “Best Dermatologist Mumbai”, “Top 10 Cosmetologist India”, etc for her evidence based expert dermatology care, affordable & reliable skin care, hair care & nail care …Read more
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