pemphigus vulgaris


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Related to pemphigus vulgaris: pemphigus foliaceus
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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

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.
References in periodicals archive ?
All clinically diagnosed cases of pemphigus vulgaris were included following a histopathological confirmation.
A multicentre randomized trial of the treatment of patients with pemphigus vulgaris with infliximab and prednisone compared with prednisone alone.
Hashimoto, "Lesional Th17 cells in pemphigus vulgaris and pemphigus foliaceus," Journal of Dermatological Science, vol.
showed similar results with 69.23% and 60.03% cases of pemphigus vulgaris respectively.
Besides the diagnosis of SOIJA, in bullous ENL other bullous disorders like pemphigus vulgaris, bullous drug eruption or bullous pemphigoid should be ruled out when there is involvement of oral mucosa and acantholytic cells are found on a Tzanck test.
The stage " Harry and Stein broke up in the 1990s, but have continued t work together and she took a break from music for three years in the mid-1980s to care for him when he became seriously ill with a rare autoimmune disease called Pemphigus Vulgaris.
singer on year " Harry and Stein broke up in the 1990s, but have continued to work together and she took a break from music for three years in the mid-1980s to care for him when he became seriously ill with a rare autoimmun disease called Pemphigus Vulgaris.
Paraneoplastic pemphigus is an autoimmune mucocutaneous disease which can imitate dermatologic diseases, like pemphigus vulgaris, erythema multiforme, erosive lichen planus and associated with lypmhocytic originated diseases.
Nursing diagnoses in pemphigus vulgaris: A case study.
Only between one and five people in a million in the country will get pemphigus vulgaris (PV) each year.
Pemphigus Vulgaris (PV) and Mucous Membrane Pemphigoid (MMP) are chronic autoimmune blistering diseases that destroy proteins essential in holding together skin and mucosal cells.