Pemphigus is a group of rare autoimmune skin disorders characterized by the
development of blisters in the outer layer of the skin (epidermis) and mucous
membranes (thin moist layers that line the body's internal surfaces). The
location and type of blisters vary according to the type of Pemphigus. If left
untreated Pemphigus can be a serious illness. Mostly misdiagnosed
If you have had chemical exposure, accidents, injury, surgery, arthritis,
then pemphigus and
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Blisters in the outer layer of the skin are common to all types of Pemphigus.
Blisters develop due to the destruction of the "cement" that holds cells
together (epidermal acantholysis) resulting in the separation of cells from one
another. Soft (flaccid) blisters generally occur on the neck, scalp, mucous
membranes, and/or underarm (axillary) and groin areas (inguinal). Most patients
with Pemphigus have deposits of IgG (an immune system antibody that defends
against foreign substances) around the blistered areas (in the epidermal cells
called keratinocytes). Antiepidermal antibodies directed against skin cells are
typically present in the fluid of the blisters. The diagnosis of Pemphigus
requires microscopic examination of cells in the blisters as well as detection
of the IgG antibodies that characterize this disease.
Pemphigus Vulgaris is the most common form and may begin with isolated blisters
on the scalp, and then in the mouth. These may persist for several months and
may be followed by blistering of the esophagus, nose, rectum, and/or the
membranes that line the inner surfaces of the eyelids (conjunctiva). The
blisters are soft; they break easily and heal poorly. Pressure on the borders of
blisters causes them to spread. Pressure on normal-looking skin can cause it to
blister (Nikolsky sign) in people with Pemphigus Vulgaris. If left untreated,
Pemphigus Vulgaris may cause life-threatening complications.
Pemphigus Vegetans is a variation of Pemphigus Vulgaris. The blisters are
fast-growing and have large (hypertrophic) lesions that are usually located in
the groin (inguinal) and armpit (axillary) areas.
Pemphigus Foliaceus is less severe and a less common form of the disorder. Soft
blisters typically occur close to the surface of the skin. When they break, they
ooze and become crusty, scaly, and susceptible to infection. Blisters may occur
on the scalp, face, upper chest, and back; the mucous membranes are usually not
affected. Small, horny plugs attached to the undersurface of the affected skin
also may be seen.
Another type of Pemphigus Foliaceus occurs in South America, particularly Brazil
and Colombia, and is called Fogo Selvagem.
Fovo Selvagem (Pemphigus)
When individuals have symptoms of both Pemphigus Foliaceus and Systemic Lupus
Erythematosus, they are said to have Pemphigus Erythematosus. Lupus, also known
as SLE, is an inflammatory disease of connective tissue. (For more information
on this disorder, choose "Lupus" as your search term in the Rare Disease
Pemphigus may also occur as a result of an adverse reaction to certain drugs
such as d-penicillamine and rifampin; symptoms usually resemble those of
Pemphigus Foliaceus rather than Pemphigus Vulgaris. Some research suggests that
Pemphigus Herpetiformis is a subtle form of Pemphigus with its own
characteristic blisters. However, blisters that form during a relapse may
resemble those of Pemphigus Foliaceus.
In Benign Familial Pemphigus (Hailey-Hailey Disease), recurrent blisters are
seen primarily on the neck, groin, and armpits. Blisters may recur because of
sweating, skin infections, and exposure to extreme heat and/or ultraviolet
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Posted on 12/1997
From the AMA
Complete Guide to Women's Health