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.
Miller-Keane Encyclopedia and Dictionary of Medicine, Nursing, and Allied Health, Seventh Edition. © 2003 by Saunders, an imprint of Elsevier, Inc. All rights reserved.

pem·phi·gus fo·li·a·'ceus

a generally chronic form of pemphigus, rarely affecting mucosal surfaces, in which extensive exfoliative dermatitis, with no perceptible blistering, may be present in addition to the bullae; serum autoantibodies induce bullae and crusted acantholytic superficial epidermal lesions.
Farlex Partner Medical Dictionary © Farlex 2012

pem·phi·gus fo·li·a·ce·us

(pem'fi-gŭs fō-lī-ā'shē-ŭs)
A generally chronic form of pemphigus in which extensive exfoliative dermatitis may be present in addition to the bullae.
Medical Dictionary for the Health Professions and Nursing © Farlex 2012
References in periodicals archive ?
Patients excluded from ABSIS assessment were defaulters in phase I who discontinued the treatment before 6months, patients currently in phase I, but not yet completed 6 months, 2 cases of pemphigus foliaceus and 6 patients who were started on dexamethasone pulse therapy
Coincidence of annular pustular psoriasis, pemphigus foliaceus, and leukocytoclastic vasculitis associated with chronic cholecystitis.
Studies show that there are possible environmental agents in the involvement of Endemic Pemphigus Foliaceus. One of them is the relationship with the sting of black flies called borrachudos.
Histopathology of skin revealed exfoliative lesion with superficial acantholysis of epidermis along with acantholytic process in external root sheath of the hair follicle and collection of neutrophils suggestive of Pemphigus foliaceus (Fig.
Sampaio et al., "Brazilian pemphigus foliaceus autoantibodies are pathogenic to BALB/c mice by passive transfer," Journal of Investigative Dermatology, vol.
Pemphigus foliaceus and oral lichen planus in a patient with systemic lupus erythematosus and thymoma.
Human autoantibodies against a desmosomal core protein in pemphigus foliaceus. J Exp Med 1984;160(5):1509-18.
Treatment of pemphigus vulgaris and pemphigus foliaceus. Expert Rev Dermatol 2009; 4(5): 469--481.
Out of 22 cases, 18 were of pemphigus vulgaris (81.81%), 2 were of pemphigus erythmatosus (9.09%), 2 cases were of pemphigus foliaceus (9.09%).