pemphigus vegetans

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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 ve·g'e·tans

1. a rare, verrucous form of pemphigus vulgaris in which vegetation develops on the eroded surfaces left by ruptured bullae; new bullae continue to form; Synonym(s): Neumann disease
2. a chronic benign vegetating form of pemphigus, with lesions commonly in the axillae and perineum; spontaneous remissions and occasionally permanent healing to occur. Synonym(s): Hallopeau disease

pemphigus vegetans

A rare form of pemphigus vulgaris which consists of a group of blistering dermopathies that begin as either pemphigus vulgaris (Neumann type, which has antibodies against 130 and 85 kD proteins), or as suprabasal acantholytic clefts (Hallopeau type, which has antibodies to the 130 kD protein), accompanied by intraepidermal eosinophils and eosinophil abscesses, which heal with verrucous epidermal hyperplasia.

pem·phi·gus vege·tans

(pemfi-gŭs vejĕ-tanz)
1. Rare, verrucous form of pemphigus vulgaris in which vegetation develops on the eroded surfaces left by ruptured bullae; new bullae continue to form.
2. Chronic benign vegetating form of pemphigus, with lesions commonly in the axillae and perineum.

Hallopeau,

François Henri, French dermatologist, 1842-1919.
Hallopeau disease - a sterile pustular eruption of the fingers and toes. Synonym(s): pemphigus vegetans; pustulosis palmaris et plantaris
Hallopeau-Siemens syndrome

Neumann,

Isidor Edler von Heilwart, Austrian dermatologist, 1832-1906.
Neumann disease - a form of pemphigus vulgaris in which vegetations develop on the eroded surfaces left by ruptured bullae. Synonym(s): pemphigus vegetans

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.