[L. pemphigus, fr Gr. pemphix, a blister, bubble]
An acute or chronic autoimmune disease principally of adults but sometimes also of children that affects the skin and mucous membranes. Antibodies form against cellular adhesion molecules in the epidermis, causing layers of the skin to separate and blister.
SYMPTOMS AND SIGNS
Pemphigus is characterized by successive crops of bullae that appear suddenly on apparently normal skin and disappear, leaving pigmented spots. Characteristic is a positive Nikolsky sign: when pressure is applied to an area as if trying to push the skin parallel to the surface, the epidermis will detach from the lower layers.
Diagnosis is by dermatological examination of the appearance and distribution of the skin lesions. Because the lesions can affect the eyes and mucous membranes of the oral cavity, pemphigus is also commonly diagnosed by otolaryngologists, periodontists, oral and maxillofacial surgeons, and ophthalmologists. Intraorally pemphigus resembles the more common diseases lichen planus and mucous membrane pemphigoid. Definitive diagnosis requires examination of a skin or mucous membrane biopsy by a dermatopathologist or oral pathologist.
If untreated, pemphigus can be fatal from an overwhelming infection of the sores. The most common treatment is the administration of oral steroids, esp. prednisone, often in high doses. Intravenous gamma globulin (IVIG) may be useful in severe cases, esp. in paraneoplastic pemphigus. Mild cases sometimes respond to the application of topical steroids. Rituximab (a monoclonal antibody) has been found to improve otherwise untreatable severe cases of pemphigus vulgaris.
All of the therapeutic drugs may cause severe side effects, and the patient must be closely monitored. Pain is common in pemphigus and must be managed.
If the patient's mouth ulcers are painful, topical anesthetics such is lidocaine are helpful. If toothbrushing is painful or contraindicated the patient should be instructed in using a mouth rinse such as a normal saline solution or half-strength hydrogen peroxide and to avoid mouthwashes that contain alcohol. Oral lesions that do not heal within two weeks must be reported.
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