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Pemphigus

Description

Description

A disease in which a patient's own immune system attacks (autoimmune disease) their skin and mucous membranes (mouth and genitals). The skin separates from the base causing blistering. These blisters can cause open sores and lead to skin infections. Rarely these infections can become serious. The disorder can occur at any age but is more common in the middle age group. There are three types of pemphigus. Pemphigus vulgaris is the most common type and affects the skin and mucous membranes. Pemphigus foliaceus only involves the skin and paraneoplastic pemphigus is associated with cancer.

Symptoms

Symptoms

Blisters on the skin, in the mouth and on the genitals. If a skin infection occurs the patient will experience skin redness, skin swelling, increased warmth and pain.

Tests

Tests

A history and physical exam will be done. The Nikolsky sign is a test done in the office where the provider will apply skin pressure next to a blister. If the skin shears away over the blister this is a positive Nikolsky sign and suggests the patient has pemphigus. A skin biopsy can confirm the disease.

Treatment

Treatment

Treatment includes steroids and immunosuppressants. Immunosuppressants prevent the body from attacking itself. If the disease is severe a plasmapharesis may be done. A plasmapharesis is performed by removing the patient's plasma and replacing it with donor plasma.



Research

Research



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