pemphigus

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Related to benign familial pemphigus: pemphigus foliaceus, pemphigus vulgaris, familial benign chronic pemphigus

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

(pem'fi-gŭs),
1. Autoimmune bullous diseases with acantholysis: pemphigus vulgaris, pemphigus foliaceus, pemphigus erythematosus, or pemphigus vegetans.
2. A nonspecific term for blistering skin diseases.
[G. pemphix, a blister]

pemphigus

/pem·phi·gus/ (-gus)
1. a distinctive group of diseases marked by successive crops of bullae.

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.
pemphigus erythemato´sus  a chronic form in which the lesions, limited to the face and chest, resemble those of disseminated lupus erythematosus.
pemphigus folia´ceus  a chronic, generalized, vesicular and scaling eruption somewhat resembling dermatitis herpetiformis or, later in its course, exfoliative dermatitis.
pemphigus ve´getans  a variant of pemphigus vulgaris in which the bullae are replaced by verrucoid hypertrophic vegetative masses.
pemphigus vulga´ris  a rare relapsing disease with suprabasal, intraepidermal bullae of the skin and mucous membranes; invariably fatal if untreated.

pemphigus

(pĕm′fĭ-gəs, pĕm-fī′gəs)
n.
Any of several acute or chronic skin diseases characterized by groups of itching blisters.

pem′phi·gous adj.

pemphigus

[pem′figəs, pemfī′gəs]
Etymology: Gk, pemphix, bubble
an uncommon, severe disease of the skin and mucous membranes, characterized by thin-walled bullae arising from apparently normal skin or mucous membrane. The bullae rupture readily, leaving raw patches. The person loses weight, becomes weak, and is subject to major infections. Treatment with corticosteroids and other immunosuppressive medications has changed the prognosis of this disease from almost certain fatality to a controllable problem compatible with a nearly normal life. The cause is unknown. Compare pemphigoid.

pemphigus

Dermatology An autoimmune disease–pemphigus foliaceus, pemphigus vulgaris–characterized by acantholysis with bulla formation. See Anti-epidemal antibody, Paraneoplastic pemphigus, Pemphigus vulgaris.

pem·phi·gus

(pem'fi-gŭs)
1. Autoimmune bullous diseases with acantholysis: pemphigus vulgaris, pemphigus foliaceus, pemphigus erythematosus, or pemphigus vegetans.
2. A nonspecific term for blistering skin diseases.
[G. pemphix, a blister]

pemphigus

A rare but serious autoimmune skin disease featuring blisters on the skin and mucous membranes of the mouth and sometimes the nose. These blisters may be induced by minor pressure and rupture easily, leaving raw areas that readily becoming infected. Pemphigus is caused by IgG autoantibodies directed against cell surface antigens (probably desmogleins) on keratinocytes which lose mutual cellular adhesion. Widespread pemphigus can lead to serious functional skin loss and life-threatening infection. Treatment is with corticosteroid or other immunosuppressant drugs. These often have to be given for long periods. A common pathophysiological mechanism is present in pemphigus, bullous impetigo and the staphylococcal scalded skin syndrome.

Pemphigus

An autoimmune disorder in which the immune system produces antibodies against specific proteins in the skin and mucous membrane. These antibodies produce a reaction that leads to a separation of skin cells.

pem·phi·gus

(pem'fi-gŭs)
Autoimmune bullous diseases with acantholysis: pemphigus vulgaris, pemphigus foliaceus, pemphigus erythematosus, or pemphigus vegetans.
[G. pemphix, a blister]

pemphigus (pem´figus, pem-fī´gus),

n a rare, serious skin disease of unknown cause characterized by the development of bullae on the skin and mucous membrane. See also bulla and sign, Nikolsky's.
Enlarge picture
Pemphigus.
pemphigus, acute disseminated,
n a serious disease of unknown cause, temporarily controlled by the administration of corticosteroids. Manifested by bullous formation on the skin and mucous membranes. Desquamation of the epithelium exposes a raw, burning, oozing submucosa. Adequate nutritional status is difficult to maintain; secondary infection is common; with progressive debility, pneumonia is common and is usually the cause of death.

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.
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