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Blistering disorders

Blistering is often seen with skin


disease. It is found with common
dermatoses such as acute contact
dermatitis, herpes simplex, herpes
zoster and bullous impetigo, and it
also occurs after insect bites, burns
and friction or cold injury.
Cont...

The type of blister depends on the level of


cleavage: subcorneal or intraepidermal
blisters rupture easily, but subepidermal ones
are not so fragile The primary bullous
disorders, dealt with here, are rare but
important.
Pemphigus

Pemphigus is an uncommon, severe and


potentially fatal autoimmune blistering
disorder affecting the skin and mucous
membranes.
Causes

Pemphigus is an autoimmune disorder. It's


not contagious. In most cases, it's unknown
what triggers the disease. Normally, your
immune system attacks foreign invaders,
such as harmful viruses and bacteria. But in
pemphigus, your immune system
mistakenly produces antibodies that attack
healthy cells in your skin and mucous
membranes.
Cont...

Rarely, pemphigus develops as a side effect of


medications, such as certain blood pressure
drugs. This type of pemphigus usually
disappears when the medicine is stopped.
Cont...
Ninety per cent of patients have
circulating IgG autoantibodies,
detectable in the serum by indirect
immunofluorescence.
Pemphigus is associated with other
organ-specific autoimmune disorders
such as myasthenia gravis.
Clinical presentation
Pemphigus is characterized by
blisters on your skin and mucous
membranes. The blisters rupture
easily, leaving open sores.
Cont...

Oral erosions signal the onset of


pemphigus vulgaris in 50% of
patients and often precede
cutaneous blistering by months.
Flaccid superficial blisters
develop over the scalp, face, back,
chest and flexures.
Cont...
Untreated, the blistering is
progressive and, prior
to the introduction of steroids,
Three out of four patients died
within 4 years, usually from
uncontrolled fluid and protein loss
or secondary infection.
Management
Treatment usually begins with
medications that are intended to
reduce signs and symptoms and
prevent complications. It's
generally more effective when it
begins as early as possible.
Treatment may also involve a
hospital stay. Pemphigus may be
life- threatening.
Cont...

Corticosteroids. For people


with mild disease, corticosteroid
cream may be enough to control
it. For others, the mainstay of
treatment is corticosteroids, such
as prednisone pills.
Cont...

Antibiotics, antivirals and


antifungal medications. These
may be used to control or prevent
infections.
Cont...

Systemic steroids and other


immunosuppressive agents are
required. Prednisolone is given
initially in a high dose (100-300
mg daily),
Cont...
Once blistering is controlled, the
steroid dosage can be lowered.
Treatment usually needs to be
continued for years, although
remission occasionally occurs.
Mortality and morbidity are now
more likely to be due to side
effects of the steroid and
immunosuppressive therapy than
to the disease itself.
Pemphigoid

Pemphigoid is a chronic and not


uncommon blistering eruption of
the Elderly.
Causes

The cause of bullous pemphigoid is


not well-understood. The blisters
occur because of a malfunction in
your immune system.
Cont…

Your body's immune system


normally produces antibodies to
fight bacteria,viruses or other
potentially harmful foreign
substances. For reasons that are
not clear, the body may develop
an antibody to a particular tissue
in your body.
Clinical presentation

Bullous pemphigoid usually


affects the elderly. Tense large
blisters arise on red or normal-
looking skin, often of the limbs,
trunk and flexures .
Oral lesions occur in only 10% of
cases.
Cont…
Pemphigoid is sometimes
localized to one site, often the
lower leg
Cont…

The skin around the blisters may


appear normal, reddish or darker
than usual. Some people with
bullous pemphigoid develop an
eczema or hive-like rash rather
than blisters.
Cont...
In most cases, the blisters appear
on the lower abdomen, groin,
upper thighs and arms.
Bullous pemphigoid usually
affects the elderly.
Management

The goals of bullous pemphigoid


treatment are to help the skin heal
as quickly as possible and relieve
itching. Your doctor will likely
prescribe a combination of drugs
that inhibit immune system
activities that cause inflammation.
These drugs may include:
Cont...
Corticosteroids. The most
common treatment is prednisone,
which comes in pill form. But
long-term use can increase your
risk of weak bones, diabetes, high
blood pressure, high cholesterol
and cataracts. Corticosteroid
ointment can be rubbed on your
affected skin and causes fewer
side effects
Cont..

Other drugs that fight


inflammation. Other drugs with
anti-inflammatory properties may
be used alone or with
corticosteroids. Examples include
methotrexa a drug; tetracycline,
an antibiotic; and dapsone a
leprosy treatment.
pompholyx

Pompholyx is a form of
hand/foot eczema characterised
by vesicles or bullae (blisters). It is
a form of vesiculardermatitis of
hands and feet, also called
vesicular endogenous eczema, and
may be the same condition as
dyshidrotic eczema
causes pompholyx

Pompholyx is multifactorial. In
many cases it appears to be
related to sweating, as flares often
occur during hot weather, humid
conditions, or following emotional
upset.
Clinical features

Pompholyx presents as recurrent


crops of deep-seated blisters on
the palms and soles. They cause
intense itch and/or a burning
sensation.

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