Pemphigus vulgaris is a rare autoimmune disease where antibodies damage skin and mucous membrane cells, causing painful blisters in the mouth, throat, eyes, genitals and other areas. It is caused when antibodies bind to proteins in the skin that hold cells together. A dermatologist can diagnose it through examination of blisters and skin biopsies. Treatment focuses on suppressing the immune system with corticosteroids and other medications to prevent new blisters and allow healing.
Pemphigus vulgaris is a rare autoimmune disease where antibodies damage skin and mucous membrane cells, causing painful blisters in the mouth, throat, eyes, genitals and other areas. It is caused when antibodies bind to proteins in the skin that hold cells together. A dermatologist can diagnose it through examination of blisters and skin biopsies. Treatment focuses on suppressing the immune system with corticosteroids and other medications to prevent new blisters and allow healing.
Pemphigus vulgaris is a rare autoimmune disease where antibodies damage skin and mucous membrane cells, causing painful blisters in the mouth, throat, eyes, genitals and other areas. It is caused when antibodies bind to proteins in the skin that hold cells together. A dermatologist can diagnose it through examination of blisters and skin biopsies. Treatment focuses on suppressing the immune system with corticosteroids and other medications to prevent new blisters and allow healing.
Pemphigus vulgaris is a rare autoimmune disease where antibodies damage skin and mucous membrane cells, causing painful blisters in the mouth, throat, eyes, genitals and other areas. It is caused when antibodies bind to proteins in the skin that hold cells together. A dermatologist can diagnose it through examination of blisters and skin biopsies. Treatment focuses on suppressing the immune system with corticosteroids and other medications to prevent new blisters and allow healing.
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PEMPHIGUS VULGARIS
BY :- Parth Chauhan (6) B1
Raj Gundaniya (11) B1 Overview • Pemphigus is a rare group of autoimmune diseases. It causes blisters on the skin and mucous membranes throughout the body. It can affect the mouth, nose, throat, eyes, and genitals. Pemphigus vulgaris is the most common type of pemphigus. • Pemphigus vulgaris is the most common type of a group of autoimmune disorders called pemphigus. Each type of pemphigus is characterized by where the blisters form. Pemphigus vulgaris affects the mucous membranes, which are found in areas including the: • mouth • throat • nose • eyes • genitals • lungs Symptoms The symptoms of pemphigus vulgaris include:
• painful blisters that start in the mouth or skin areas
• skin blisters near the surface of the skin that come and • gooozing, crusting, or peeling at the blister site Causes • Pemphigus is an autoimmune disorder. Normally, your immune system produces antibodies to fight off harmful invaders, such viruses and bacteria. But in pemphigus, the body produces antibodies that damage cells of your skin and mucous membranes. • The keratinocytes are cemented together at unique sticky spots called desmosomes. In pemphigus vulgaris, immunoglobulin type G (IgG) autoantibodies bind to a protein called desmoglein 3 (dsg3), which is found in desmosomes in the keratinocytes near the bottom of the epidermis. The result is the keratinocytes separate from each other, and are replaced by fluid (the blister). • About 50% of patients with pemphigus vulgaris also have anti-dsg1 antibodies. Risk factors • Your risk of pemphigus increases if you're middle-aged or older. The condition tends to be more common in people of Middle Eastern or Jewish descent. • Signs of an infected blister can include: • the skin becoming painful and hot • yellow or green pus in the blisters • red streaks leading away from the blisters Diagnosed • A dermatologist will conduct a physical examination of your skin blisters. They will look for an indicator of the condition called Nikolsky’s sign. A positive Nikolsky’s sign is when your skin shears off easily when the surface is wiped sideways with a cotton swab or a finger. • Direct immunofluorescence of a skin biopsy taken of normal skin near a bulla shows a linear deposition of C3 and IgG along the basement membrane zone. • Indirect immunofluorescence of the blood shows linear IgG along the blister roof on salt-split skin (Many patients do not have detectable circulating antibodies). • An enzyme-linked immunosorbant assay (ELISA) for BP 180 and BP 230 is available. Treatment The main aim of treatment is to heal the blisters and prevent new ones forming. • Steroid medication (corticosteroids) plus another immunosuppressant medication are usually recommended. These help stop the immune system damaging healthy tissue. • Common corticosteroids include prednisone or prednisolone. A high dose is usually needed to control the condition at first. • You may need to take supplements such as calcium and vitamin D, eat a low-sugar diet, or take other medications to treat these side effects. Additional medications that suppress the immune system. • azathioprine • mycophenolate mofetil