Pemphigus (rare skin disorder)

Description A disease in which ...

Description

A disease in which a person's immune system attacks (autoimmune disease) the skin and mucous membranes (mouth and genitals). The outer part of the skin separates from the inner part of the skin in various places, causing blisters to form. 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. Paraneoplastic pemphigus involves the skin, and is associated with cancer.

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

Common tests used for diagnosis and treatment

Workup:
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.

Specialists:
Internal Medicine, Dermatology, Pediatrics, Family Practice, Pediatric Dermatology, Telemedicine Dermatology

Treatment

Treatment includes steroids and immunosuppressants. Both treatments quiet the overactive immune system, and prevent the body from attacking itself. If the disease is severe, plasmapheresis may be done. Plasmapheresis is performed by removing the patient's plasma (blood fluid from which blood cells have been removed) and replacing it with donor plasma.