pemphigus (redirected from benign familial pemphigus)

pemphigus /pem·phi·gus/ (-gus)

1. a distinctive group of diseases marked by successive crops of bullae.

2. pemphigus vulgaris.

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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.

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pem·phi·gus (pmf-gs, pm-fgs)

n.

Any of several acute or chronic skin diseases characterized by groups of itching blisters.

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pemphi·gous adj.

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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.

Mentioned in: Corticosteroids, Systemic

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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.

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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.

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.

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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.

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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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pemphigus

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