Pemphigus occurs worldwide, in both males and females of all ethnic backgrounds. The most common form, pemphigus vulgaris, occurs more often in individuals of Ashkenazi Jewish descent and those of Mediterranean origin. On average, the disease begins around age 50-60 years, but pemphigus may occur in children and older adults as well. Some people have a genetic predisposition to developing pemphigus. It may also occur in individuals with other autoimmune disorders such as myasthenia gravis and thyroid cancer.
Types of pemphigus
The three main types of pemphigus are pemphigus vulgaris, pemphigus foliaceus, and paraneoplastic pemphigus.
Pemphigus vulgaris accounts for about 70% of cases of pemphigus. In 50-70% of individuals with pemphigus vulgaris, painful sores develop in the mouth and may spread into the upper part of the throat. Painful sores also appear in the skin. Firm pressure on normal-looking skin produces a tear or sore (called Nikolsky’s sign). Pemphigus vulgaris does not affect any of the internal organs.
Pemphigus foliaceus occurs less often than pemphigus vulgaris. Blisters or sores usually first appear on the face and scalp, and may spread to the chest and back, but do not occur in the mouth. The blisters are often itchy but not as painful as in pemphigus vulgaris.
Paraneoplastic pemphigus is the least common, but most serious, form of pemphigus. Most cases occur in individuals who have already been diagnosed with cancer. Painful sores form in the mouth, in the throat, and on the lips, as well as on the skin. Paraneoplastic pemphigus may also affect the lungs.
Diagnosis and treatment
The appearance and location of the blisters and sores can suggest the diagnosis of pemphigus. A sample of the cells from the edge of a blister can help determine which type of pemphigus is present. Special tests called indirect and direct immunofluorescence can aid in the diagnosis.
The goal of treatment in pemphigus is to decrease blister formation and promote healing of existing sores. Treatment will depend upon what type of pemphigus is present and what other conditions an individual has. In general, treatment will try to reduce autoantibody production and the inflammatory response in the body. Corticosteroid medications such as prednisone and immunosuppressive drugs such as azathioprine (Imuran) may be used. Care of existing sores includes gentle cleansing and the use of topical creams to promote wound healing.
Information for this article was taken from:
International Pemphigus Foundation. What is Pemphigus?
Zeina, B., & Ali, M. (2005). Pemphigus vulgaris. eMedicine, accessed at http://www.emedicine.com/derm/topic319.htm
