Pemphigus

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pemphigus

[′pem·fə·gəs]
(medicine)
An acute or chronic disease of the skin characterized by the appearance of bullae, which develop in crops or in continuous succession.

Pemphigus

 

a chronic disease characterized by a crop of blisters, or bullae, having a flaccid covering and serous-hemorrhagic contents; the blisters form on the apparently intact skin or mucosa of the oral cavity, larynx, eyes, and genitals. The blisters rapidly enlarge and multiply, bursting to form extensive ulcerated surfaces. The patient’s general condition is disturbed; there is general weakness and elevation of body temperature. Infection of the mouth and larynx makes food intake difficult.

The causes of pemphigus have not been conclusively determined. The disease usually afflicts middle-aged and elderly persons. The mechanism of formation of pemphigus vulgaris is acantholysis, a type of degenerative change in epidermal cells. It involves the dissolution of the intercellular bridges, degenerative change of the nuclei, and loss of part of the cell protoplasm. As a result, communication between the layers of epidermis is disrupted. In other forms of pemphigus, the blisters form as a result of an inflammatory process.

Pemphigus vulgaris progresses gradually, and cachexia develops. The prognosis for persons suffering from other forms of pemphigus is relatively favorable. Treatment includes the ingestion of hormonal preparations, antimicrobial agents, or analgesics. Disinfecting solutions may be applied externally.

S. S. KRIAZHEVA

References in periodicals archive ?
Paraneoplastic pemphigus: a report of three cases including one long-term survivor.
The mechanism of respiratory failure in paraneoplastic pemphigus. N Engl J Med 1999; 340:1406-1410.
Paraneoplastic pemphigus associated with bronchiolitis obliterans.
Paraneoplastic pemphigus associated with Castleman tumor, myasthenia gravis and bronchiolitis obliterans.
(27.) Zhu X, Zhang B: Paraneoplastic pemphigus. J Dermatol 2007;34:503-11.
(28.) Kimyai-Asadi A, Jih MH: Paraneoplastic pemphigus. Int J Dermatol 2001;40:367-72.
(29.) Menenakos C, Braumann C, Hartmann J, Jacobi CA: Retroperitoneal Castleman's tumor and paraneoplastic pemphigus: report of a case and review of the literature.
(30.) Camisa C, Helm TN: Paraneoplastic pemphigus is a distinct neoplasia-induced autoimmune disease.
(31.) Mutasim DF, Pelc NJ, Anhalt GJ: Paraneoplastic pemphigus. Dermatol Clin 1993;11:473-81.
Immune dysfunction in the elderly encourages the development of several autoimmune disorders: bullous and benign mucous membrane pemphigoid, paraneoplastic pemphigus, and the potentially fatal pemphigus vulgaris.8
Pemphigus consists of 3 primary subsets (pemphigus vulgaris, pemphigus foliaceus, and paraneoplastic pemphigus) as well as other uncommon variants.
(4.) Zhu X, Zhang B: Paraneoplastic pemphigus. Journal of Dermatology 2007;34:503-11.

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