Erbil polytechnic university

Erbil health technical collage

Physiotherapy department
2020_2021
2nd Stage

PEMPHIGUS

PREPARED BY : SUPERVISED BY:

Nawshirwan Fazil dr.Suhair Abdullah

Abdulrahman Jabar
Zaid masoud
 Content

o Abstract

o Introduction :

• Types pf pemphigus
• symptoms
• Causes
• Who is at risk
• How it is diagnosed
• How it gets treated
• Complications

o Conclusion

o Review of related study

o Recommendation

o references
Abstract :
Pemphigus vulgaris is a chronic autoimmune mucocutaneous disease that initially
manifests in the form of intraoral lesions, which spread to other mucous
membranes and the skin. The etiology of pemphigus vulgaris is still unknown,
although the disease has attracted considerable interest. The pemphigus group of
disease is characterized by the production of autoantibodies against intercellular
substances and is thus classified as autoimmune diseases. Most patients are
initially misdiagnosed and improperly treated for months or even years[1]

Introduction
Pemphigus vulgaris is a rare autoimmune disease that causes painful blistering on
the skin and mucous membranes. If you have an autoimmune disease, your
immune system mistakenly attacks your healthy tissues.
Pemphigus vulgaris is the most common type of a group of autoimmune disorders
called pemphigus. Each type of pemphigus is characterized by where the blisters
form.

Pemphigus vulgaris affects the mucous membranes, which are found in

areas including the:

• mouth
• throat
• nose
• eyes
• genitals
• lungs

This disease usually starts with blisters in the mouth and then on the skin. The
blisters sometimes affect the membranes of the genitals.

Pemphigus vulgaris can be dangerous. Treatment is essential, and typically

involves the use of corticosteroids to suppress the immune system. The condition
can cause serious complications if it isn't treated. Some of these complications can
be fatal.

The death rate from this disease had averaged 75 percent before corticosteroids
were introduced in the 1950s. This has improved dramatically with today's
treatments.[2]
 Symptoms

Signs and symptoms can also show up quickly. Blisters can appear
suddenly and spread. Widespread pemphigus can be life threatening. It
can turn an otherwise healthy person into one who is extremely sick,
incredibly tired, and in pain.[3]

The symptoms of pemphigus vulgaris include:

• painful blisters that start in the mouth or skin areas

• skin blisters near the surface of the skin that come and go
• oozing, crusting, or peeling at the blister site[2]

[3]

Other signs and symptoms of pemphigus

 Pain
 Fatigue
 Weakness
 Light sensitivity
 Eye problems[3]
 causes of pemphigus
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.
Very rarely, certain medications can cause pemphigus vulgaris.
These drugs include:

• penicillamine, which is a chelating agent that removes certain materials from

the blood

• ACE inhibitors, which are a type of blood pressure medication[2]

Who is at risk for pemphigus
Pemphigus vulgaris isn't contagious and cannot be transmitted from one person to
another. It also doesn't appear to be transmitted from parent to child. However, a
person's genes can put them at a higher risk for the condition. If your parents or
other family members had or have the condition, you're more likely to develop it.

Pemphigus vulgaris can affect people of all races, genders, and ages. However, the
condition is more common in the following groups:

• people of Mediterranean descent

• eastern European Jews

• people who live in the rainforests in Brazil

• middle-aged and older adults

How is the pemphigus

diagnosed
A dermatologist will conduct a physical examination of your skin blisters. They
will look for an indicator of the condition called Nikolsky's sign. A positive
Nikolsky's sign is when your skin shears off easily when the surface is wiped
sideways with a cotton swab or a finger.

Your doctor may then take a biopsy of the blister, which involves removing a
piece of tissue for analysis, and looking at it under a microscope to confirm the
diagnosis. The biopsy may be treated in the laboratory with chemical substances
that help your doctor find the abnormal antibodies. Your doctor can use this
information to determine the type of pemphigus [2]

Types of pemphigus
The different types of pemphigus are diagnosed based on the location of the
blisters. They include:

• Pemphigus vulgaris
Pemphigus vulgaris is the most common type of pemphigus in the Unites States.
Blisters usually first appear in the mouth. The blisters don't itch. They can be
painful. Blisters may then appear on the skin and sometimes on the genitals.

• Pemphigus foliaceus
Pemphigus foliaceus doesn't cause blisters in the mouth. The blisters first appear
on the face and scalp. Blisters then appear on the

• Pemphigus vegetans
Pemphigus vegetans causes blisters that appear on the groin, under the arms, and
on the feet.

• Paraneoplastic pemphigus
A very rare type of pemphigus that occurs in people with some cancers is called
paraneoplastic pemphigus. The blisters and sores may appear in the mouth, on the
lips, and on the skin. This type may also cause scars on the eyelids and eyes. It can
also cause lung problems. [2]
How is the pemphigus treated
Treatment is aimed at reducing pain and symptoms and preventing complications
like infection. It involves one or more drugs and other methods. It may include any
of the following:

 Corticosteroids and immune-suppressing drugs

A high dose of corticosteroids is the core treatment for the condition.

Common corticosteroids include prednisone or prednisolone. A high dose
is usually needed to control the condition at first.

These drugs have many side effects, including:

 an increased likelihood of becoming infected

 osteoporosis
 cataracts
 glaucoma
 increased blood sugar 
 diabetes
 a loss of muscle mass
 stomach ulcers
 water retention [2]
You may need to take supplements such as calcium and vitamin D, eat a
low-sugar diet, or take other medications to treat these side effects
Once the blisters are under control, the dose may be lowered to the lowest
level needed to prevent new blisters and to keep the side effects at a
minimum. A corticosteroid cream can be used directly on the blisters as
well.

To help keep the dose of corticosteroids low, your doctor may prescribe
additional medications that suppress the immune system. These include:

 azathioprine
 mycophenolate mofetil
 methotrexate
 cyclophosphamide
 rituximab

 Antibiotics, antivirals, and antifungals

Any of these may be prescribed to prevent other infections .

 Intravenous (IV) feeding

If your mouth ulcers are severe, you may not be able to eat without pain.
You may need to be fed through your veins. This involves using an
intravenous (IV) connection.
  Plasmapheresis

In very severe cases, a person may undergo a procedure known as

plasmapheresis. This procedure is intended to remove the antibodies
attacking the skin from the blood. During this procedure, the plasma, or
fluid part of the blood, is removed by a device and replaced with donated
plasma. This treatment can be very expensive.

 Wound management

If the blisters are severe, you may need to stay in the hospital to get wound
treatment. This treatment is similar to what’s given for severe burns. You
may need to receive IV fluids and electrolytes if you’ve lost too much fluid
through oozing of the blisters.

The treatment for the blisters may also include:

 numbing lozenges for mouth blisters

 soothing lotions
 wet dressings
 pain medications
 soft-food diets
 avoidance of spicy or acidic foods that may irritate the blisters
 avoidance of too much sun exposure
If the blisters in your mouth keep you from brushing or flossing your teeth,
you may need special oral health treatment to prevent gum disease and
tooth decay. See your dentist to ask them about oral care.[2]

complications of pemphigus

The complications of pemphigus vulgaris can be fatal and severe.

They may include:

 skin infections
 sepsis, or the spread of infection through the bloodstream
 dehydration
 side effects of medication [2]
 long-term outlook for
people with pemphigus

If left untreated, pemphigus vulgaris can be life-threatening. The most

common cause of death is a severe secondary infection.

Pemphigus vulgaris is a lifelong condition. It cannot be cured. However,

most people go into remission after receiving corticosteroids.
Improvement is usually noticed within days of starting the
corticosteroids.

The blisters will heal slowly, especially the ones in the mouth. On
average, the blisters stop forming in two to three weeks. Healing of the
blisters takes six to eight weeks on average. However, full healing can
sometimes take years. Some individuals may need to stay on a low dose
of the medication for life. [2]
 conclusion
Pemphigus, especially some types, is a life-threatening disease and has a mortality
risk. Therefore, the diagnosis should be made as soon as possible, and the
treatment should be started.
Today, a better understanding of the role of immunological dysregulation in the
pathogenesis will also cause offering newly targeted therapeutical agents in the
treatment of pemphigus.

The review findings emphasize the importance of early diagnosis, early initiation
of treatment, and use of steroid-sparing adjuvants to allow a reduced total dose
and duration on corticosteroids. Also highlighted is the need for more RCTs. [4] [5]
 Review of related study

All-cause and cause-specific mortality among patients with pemphigus compared

with the general population is yet to be established.

This study investigated overall mortality and cause-specific mortality in a large

immunopathologically validated cohort of patients with pemphigus. Mortality of
patients with pemphigus was compared with age- and gender-matched control
subjects in the general population.

All-cause and cause-specific standardized mortality ratios (SMRs) were

estimated. The study cohort included 245 patients newly-diagnosed with
pemphigus between January 1990 and June 2016, contributing 2,679.4 person-
years of follow-up.

Overall, 48 deaths were observed du[ring a mean follow-up period of 10.9 ± 8.1
years, which was more than twice the number expected (SMR 2.4; 95%
confidence interval (95% CI) 1.82-3.20). The SMRs for death due to infections
(22.6; 95% CI 13.6-35.3), namely pneumonia (25.7; 95% CI 11.7-48.8) and
septicaemia (8.6; 95% CI 1.7-25.0), and due to cardiovascular diseases (2.8; 95%
CI 1.0-6.0) were significantly higher than expected.

Overall mortality among patients with pemphigus is 2.4-times greater than for the
general population, mainly due to infections.[6]
We did a research asking 100 people about pemphigus disease
%77 of the people were female and %23 were male
Nearly %66 did not have the disease only %7 were certainly had the
disease others were not sure if they had it or had not notice it
which means this disease fortunately have a low rate in Kurdistan

Female & male percentage :

Their age :
Do they got the disease :

Their complications :
 Recommendation
In the last 20 consecutive patients with pemphigus or all patients seen in the last
12 months (if fewer than 20), is there dear docu-mentation of

 Measurement of baseline parameters prior to starting treatment.

as a minimum this should include

 Appropriate investigations to establish diagnosis. As a minimum this

should include

 Evidence of appropriate drug monitoring. For patients on corticosteroids,

as a minimum this should include regular measurements of or
documentation of the management of pemphigus

 Adherence to guidelines for prophylaxis and management of steroid-

induced osteoporosis
  Use of objective disease-scoring methodologies to assess clinical
outcomes, for example PDAI, ABSIS or the Oral Disease Severity Score

The usual audit recommendation of 20 cases per department is to reduce

variation in the results due to a single patient, and to allow benchmarking
between different units. How-ever, departments unable to achieve this
recommendation may choose to audit all
cases seen in the preceding 12 months
[7]

References

[1] https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3180831/

[2] https://www.healthline.com/health/pemphigus-vulgaris#causes

[3] https://www.aad.org/public/diseases/a-z/pemphigus-symptoms

[4] https://onlinelibrary.wiley.com/doi/abs/10.1111/bjd.14463

[5] https://link.springer.com/article/10.1007/s12325-016-0343-4

[6] https://scholar.google.com/scholar?
hl=en&as_sdt=0%2C5&q=articles+about+pemphigus+in+2017&oq=#d=gs_qabs&
u=%23p%3DnpXZVZ2zE_sJ

[7] https://onlinelibrary.wiley.com/doi/full/10.1111/bjd.15930