pemphigus
(pem'fĭ-gŭs 
)
[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: 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.
Treatment: 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.
Patient Care: 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.
endemic p. foliaceus Fogo selvagem.
erythematous p.Scaling, erythematous macules and blebs of the scalp, face, and trunk. The lesions have a “butterfly” distribution over the face. The disease resembles pemphigus foliaceus.
familial benign chronic p.Hailey-Hailey disease.
ABBR: PF
Pemphigus that causes blistering of the skin of the head, neck, or trunk; the mouth is unaffected.In Western countries, PF is often associated with the recent use of certain medications, such as ACE inhibitors or antirheumatic disease modifying drugs. In the developing world, it occurs in an endemic form in certain locations.
Common symptoms of PH include blisters that may be soft, oozing, crusty, or scaly on the face, scalp, upper chest and back; itching, and pain. PF also often produces a burning sensation on affected skin.
Topical imiquimod, immunosuppressant drugs, and monoclonal antibody therapies have been used to treat all forms of pemphigus foliaceus.
Blisters should be kept clean and dry and should not be disturbed (rubbed, scratched, or popped). Affected skin should be protected from abrasion on neighboring body parts or clothing, and kept out of direct sunlight. Professional health care should be sought when blisters become painful or obviously infected, or when they form near or in the eyes, mouth, or anus.
paraneoplastic p.Pemphigus that arises together with or sometimes before malignancy, particularly B-cell lymphomas and chronic lymphocytic leukemia.
p. vegetans A localized form of pemphigus vulgaris characterized by pustules instead of bullae. Pustules are followed by warty vegetations. Prognosis is good, even before therapy with corticosteroids.
p. vulgaris The most common form of pemphigus.
Blisters develop suddenly and are round or oval, thin-walled, tense, and translucent and bilateral in distribution. The lesions have little tendency to heal, and bleed easily when they burst.
Physical examination shows flat bullae and a positive Nikolsky sign. Conclusive diagnosis is by presence of acantholytic cells in examination of a punch biopsy from the area around the lesion with direct immunofluorescent staining. The cells can also be seen on a Tzanck smear. The acantholytic cells are rounded, nucleated keratinocytes resulting from antibody mediated damage to cell adhesion protein.
Since the introduction of corticosteroids, the prognosis for this autoimmune disease is favorable, but the mortality rate is still 5% to 15%. Immunosuppressive agents (such as azathioprine or cyclophosphamide) are used with corticosteroid therapy.
Nursing consists primarily of management of the symptoms, esp. pain, and of prevention of complications, esp. infection.
SEE: photochemotherapy.
illus.illus.PEMPHIGUS