Pemphigus is a group of rare skin disorders that cause
blisters and sores on the skin or mucous membranes, such as in the mouth or on
the genitals. The two main types are pemphigus vulgaris and pemphigus
foliaceus. Pemphigus vulgaris usually starts in your mouth. It can be
painful. Pemphigus foliaceus affects the skin and tends to be more itchy than
painful. Pemphigus can occur at any age, but it's most often seen in people who
are middle-aged or older. Pemphigus is not to be confused with bullous
pemphigoid, another blistering skin condition. Usually a chronic condition,
pemphigus is best controlled by early diagnosis and treatment. Treatment may
include medications and therapies similar to those used for severe burns.
Pemphigus vulgaris
“Pemphigus” is used in a very
specific way to describe blistering disorders caused by auto antibodies against
some part of the epidermis, which lead to disruption of the intercellular
junctions (and hence bullae). Pemphigus vulgaris, not surprisingly, is the most
common type of pemphigus (“vulgar-” comes from the Latin vulgaris, meaning the
general public). It occurs primarily in adults between the ages of 30 and 60,
and is characterized by big, flaccid bullae that burst easily (in most
patients, you’ll see more ruptured, scab-covered bullae than intact ones).
Patients often present first with oral bullae and ulcerations, and later
develop bullae on the skin.
In this disease, patients have auto
antibodies against desmogleins, which disrupt the connections between the
squamous cells of the epidermis and cause very superficial, intraepidermal,
fragile bullae. If you do immunofluorescence on the skin, you’ll see a kind of
outlining of each individual epidermal cell (because there are auto antibodies
bound to the junctions between the cells). Treatment consists of
immunosuppressive agents; prognosis is variable, but many patients have a
higher than normal mortality rate.
Pemphigus foliaceus
Pemphigus foliaceus is part of the
pemphigus group of autoimmune diseases. Autoimmune diseases occur when the
body's immune system attacks healthy tissue. In pemphigus foliaceus, the immune
system damages skin cells called keratinocytes.
Pemphigus foliaceus
(PF) causes blisters, cuts, and crusty spots on
the skin. The sores can be painful and
unattractive, but PF is a fairly benign medical condition that does not
typically cause other health problems. A chronic, recurring disorder, PF cannot
be cured by medical treatment. However, some people go into spontaneous
remission, meaning they do not have symptoms for long. PF symptoms can also be
managed by treatment.
What are the symptoms of pemphigus vulgaris?
Skin erosions (sores) are
painful and can affect quality of life including disturbing sleep. Mouth
and throat lesions can interfere with eating and drinking and so cause weight
loss. Pemphigus vulgaris can also affect other mucous membranes such as the genital
area, leading to painful sexual intercourse, urination and bowel movements, the
nose causing stuffiness and blood-stained crusts; and the conjunctiva of the
eyes causing sore, red eyes (requiring assessment by an ophthalmologist).The
most common area to be affected is the inside of the mouth. This is most
commonly the first area to be affected, and skin lesions often appear later.
Other areas, as described above, are less commonly affected. The major symptom of pemphigus vulgaris is the
development of clear, soft, and painful (sometimes tender) blisters of various
sizes. In addition, the top layer of skin may detach from the lower layers in
response to slight pinching or rubbing, causing it to peel off in sheets and to
leave painful areas of open skin (erosions).
The blisters often first appear in the mouth
and soon rupture, forming painful sores (ulcers). More blisters and ulcers may
follow until the entire lining of the mouth is affected, causing difficulty
swallowing, eating, and drinking. Blisters form in the throat as well. Blisters
can form on the skin and rupture, leaving raw, painful, crusted wounds. The
person feels generally ill. Blisters may be widespread, and once ruptured, they
may become infected. When severe, pemphigus vulgaris is as harmful as a serious
burn. Similar to a burn, the damaged skin oozes large amounts of fluid and is
prone to infection by many types of bacteria.
What causes pemphigus vulgaris?
The immune system produces
proteins called antibodies. Antibodies normally attack harmful foreign
substances like bacteria and viruses. Pemphigus vulgaris occurs when the immune
system mistakenly makes antibodies against proteins in healthy skin and mucous
membranes. The antibodies break down the bonds between the cells, and fluid
collects between the layers of the skin. This leads to blisters and erosions on
the skin. The precise cause of the attack by the immune system isn’t known.
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