pemphigus vulgaris


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Related to pemphigus vulgaris: pemphigus foliaceus

pemphigus

 [pem´fĭ-gus]
any of a group of diseases characterized by successive crops of large bullae (“water blisters”). Although rare, they are serious and require prompt treatment. The cause is unknown; they seem to occur only in adults and can occur in acute or chronic form. The term is often used alone to refer to pemphigus vulgaris.

Clusters of blisters usually appear first near or inside the nose and mouth and then gradually spread over the skin of the rest of the body. When the blisters burst, they leave round patches of raw and tender skin. The skin itches, burns and gives off an offensive odor. The patient loses appetite and weight. If the disease is allowed to progress, it may cause extreme weakness, prostration and shock, accompanied by chills, sweating, fever, and often pneumonia.

The patient must be hospitalized from the beginning and given antibiotics and sometimes blood transfusions. Intense discomfort is present and the patient may need to suck anesthetic tablets to allay pain around the mouth while eating. Progress has been made in the treatment of this disease through the persistent use of cortisone, administered orally, and of the pituitary extract ACTH, administered intramuscularly. Fatalities, once fairly common, now can usually be averted. The disease is difficult to control, however, and therapy sometimes must be maintained for years to prevent continuing attacks.
benign familial pemphigus a hereditary, recurrent vesiculobullous dermatitis, usually involving the axillae, groin, and neck, with crops of lesions that regress over several weeks or months. Called also Hailey-Hailey disease.
pemphigus erythemato´sus a variant of pemphigus foliaceus in which the lesions, limited to the face and chest, resemble those of disseminated lupus erythematosus.
pemphigus folia´ceus a superficial, relatively mild and chronic form of pemphigus, usually occurring in the fourth and fifth decades of life, and characterized by the development of small flaccid bullae that rupture and crust and localized or generalized exfoliation. The lesions may be found on the scalp, face, and trunk, or they may spread to become generalized.
pemphigus ve´getans a variant of pemphigus vulgaris in which the bullae are replaced by large wartlike vegetative masses.
pemphigus vulga´ris the most common and severe form of pemphigus, usually occurring between the ages of 40 and 60, characterized by the chronic development of flaccid, easily ruptured bullae upon apparently normal skin and mucous membranes, beginning focally but progressing to become generalized, leaving large, weeping, denuded surfaces that become partially crusted over with little or no tendency to heal and that enlarge by confluence. In untreated cases, sepsis, cachexia, and electrolyte imbalance may occur and lead to death.

pem·phi·gus vul·ga·'ris

a serious form of pemphigus, occurring in middle age, in which cutaneous flaccid acantholytic suprabasal bullae and oral mucosal erosions may be localized a few months before becoming generalized; blisters break easily and are slow to heal; results from the action of autoimmune antibodies that localize to intercellular sites of stratified squamous epithelium.

pemphigus vulgaris

Etymology: Gk, pemphix, bubble; L, vulgus, common
a chronic, progressive autoimmune disease that is often fatal, characterized by the formation of bullae on otherwise normal oral mucosal membrane. Also called pemphigus chronicus.
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Pemphigus vulgaris

pemphigus vulgaris

An autoimmune intraepithelial bullous dermatosis, which primarily affects older people and is characterised by suprabasilar acantholysis and blistering of mucocutaneous surfaces, especially of the oral cavity.

Aetiology
Immune response to penicillamine and captopril; > 95% of patients have specific HLA antigens; ±50% of patients begin with oral blisters, followed by skin blistering.

DiffDx
Bullous impetigo, bullous pemphigoid, (bullous) erythema multiforme, dermatitis herpetiformis, Sweet syndrome.

pemphigus vulgaris

Dermatology An autoimmune disorder characterized by acantholysis and blistering of mucocutaneous surface primarily in older people Etiology Immune response to penicillamine and captopril; > 95% of Pts have specific HLA antigens; ±50% of Pts begin with blisters in the mouth, followed by skin

pem·phi·gus vul·ga·ris

(pem'fi-gŭs vŭl-gā'ris)
A serious form of pemphigus, occurring in middle age, in which cutaneous bullae and oral erosions may be localized a few months before becoming generalized; blisters break easily and are slow to heal; results from the action of autoimmune antibodies that localize to intercellular sites of stratified squamous epithelium.

pemphigus vulgaris

chronic, initially localized but later generalized autoimmune inflammatory disease; characterized by easily ruptured, flaccid cutaneous bullae and blood-filled blisters at the dermoepidermal junction

pemphigus

[Gr.] pemphix (blister); a group of immune-mediated diseases of the skin and mucous membranes characterized by vesicles, bullae, erosions and ulcerations; occurs in dogs, cats and horses.

pemphigus erythematosus
a form with features of both pemphigus foliaceus and lupus erythematosus; occurs in dogs and cats. There are erythematous, pustular lesions, mainly on the nose, periorbital skin and pinnae, and hypopigmentation of the planum nasale.
pemphigus foliaceus
a generalized, exfoliative, scaling disease sometimes with the formation of heavy crusts, marked hyperkeratosis of footpads, and involvement of the nail beds that may lead to loss of the nails. Occurs in dogs, cats, horses and goats.
pemphigus vegetans
a benign variant of pemphigus vulgaris, occurring only rarely in animals, in which the bullae are replaced by verrucoid hypertrophic vegetative masses.
pemphigus vulgaris
consists of shallow ulcerations with a generalized distribution and frequently involving the mucocutaneous junctions and oral mucosa.
References in periodicals archive ?
Devise corrective measures for pipeline projects by understanding Pemphigus Vulgaris pipeline depth and focus of Indication therapeutics.
Of the 3 pemphigus vulgaris specimens that were negative for IgG4 immunostain, 1 specimen did not have acantholytic lesions.
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).
Mycophenolate is effective in the treatment of pemphigus vulgaris.
These findings agree with studies demonstrating a high production of IL-1 in peripheral blood mononuclear cells from patients with active EPF lesions, (4) and with those investigating other blistering diseases, such as pemphigus vulgaris, in which the expression of proinflammatory cytokines, such as TNF-[alpha] and IL-6, around the blister was demonstrated by in situ hybridization.
Pemphigus vulgaris is the most common autoimmune bullous disease in Northwestern Romania.
In the review by Woldegiorgis and Swerlick (see page 694 of this issue), it is apparent that clinicians in the Southeast are not familiar with the clinical presentation of pemphigus vulgaris and pemphigus foliaceous since an average delay in proper diagnosis was from 4 to 6 months.
Coverage of the Pemphigus Vulgaris pipeline on the basis of therapeutic class, route of administration and molecule type.
The pathophysiological significance of non-desmoglein targets of pemphigus autoimmunity: pemphigus vulgaris and foliaceus patients develop antibodies against keratinocyte cholinergic reseptors.
Autoantibodies found in patients with pemphigus vulgaris bind Dsgl or both Dsg3 and Dsgl.
According to this new classification, pemphigus group diseases include, pemphigus vulgaris, pemphigus vegetans, pemphigus foliaceus, pemphigus erythematosus, endemic pemphigus, IgA pemphigus, pemphigus herpetiformis, paraneoplastic pemphigus, and drug-induced pemphigus.
The present Competitive Intelligence Report about p38 MAPK inhibitors used to treat inflammatory diseases such as rheumatoid arthritis, psoriasis, COPD or pemphigus vulgaris and pain, respectively, provides a competitor evaluation in the field of R&D projects with p38 MAPK inhibitors as of March 2009.