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 ?
We searched our pathologic archive and retrieved 18 specimens of pemphigus from 15 patients, including 12 cases of pemphigus vulgaris and 6 of pemphigus foliaceus.
Bastuji-Garin S, Souissi R, Blum L, et al: Comparative pidemiology of pemphigus in Tunisia and France: unusual incidence of pemphigus foliaceus in young Tnisian women.
Immunoelectron microscopy has localized both pemphigus vulgaris (desmoglein 3) and pemphigus foliaceus (desmoglein 1) antigens to the desmosomes, the most prominent cell-cell adhesion junctions in stratified squamous epithelia (12, 13).
In the second study involving patients with steroid-dependent pemphigus foliaceus, varying intervals of IVIg therapy were given at 1-2 mg/kg per cycle.
The Foundation focuses on the three main types of pemphigus, which vary in severity: pemphigus vulgaris, the most common, pemphigus foliaceus, the least severe, and paraneoplastic pemphigus, the least common and most severe.
Mahoney MG, Wang Z, Rothenberger K, Koch PJ, Amagai M, Stanley JR: Explanations for the clinical and microscopic localization of lesions in pemphigus foliaceus and vulgaris.
Endemic pemphigus foliaceus (EPF), or fogo selvagem, is a chronic autoimmune disease (1)fc characterized by the formation of intraepidermal blisters, which are attributed to the presence of immunoglobulin G4 (IgG4) and IgG1 autoantibodies against desmoglein 1 (2) that reduce the adhesion between keratinocytes.
1, Medicare will start covering IVIg treatments for certain mucocutaneous blistering diseases, including pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid, cicatricial pemphigoid, and epidermolysis bullosa acquisita.