this topic is on phemphigus vulgaris. describes in detail regarding the phemphigus, its causes, pathophysiology, management and nursing care plan

1. Phemphigus
By:
Mr. M. Sivananda Reddy

2. Definition
Pemphigus is a blistering autoimmune disease
that affects the skin and mucous membranes.

3. Phemphigus Blisters

4. Etiology:
• Autoimmune disease involving immunoglobulin G.
• Genetic factors may also play a role in its
development, with the highest incidence among those
of Jewish or Mediterranean descent.
• Usually occurs in men and women in middle and late
adulthood.
• May be associated with Penicillins and Captopril and
with Myasthenia Gravis.

5. Pathophysiology
• Pemphigus antibody is directed against a
specific cell-surface Antigen (Desmoglein) in
epidermal cells.
• A blister forms from the antigen–antibody
reaction.

6. Types
There are three types of pemphigus which vary
in severity:
• Pemphigus vulgaris
• Pemphigus foliaceus
• Paraneoplastic pemphigus.

7. • The most common form of the disorder is
pemphigus vulgaris . It occurs when antibodies
attack Desmoglein 3 (a protein that is present in
the epidermal layer of skin).
• Pemphigus foliaceus (PF) is the least severe of
the three varieties. Desmoglein 1, the protein
that is destroyed by the autoantibody

8. • The least common and most severe type of
pemphigus is paraneoplastic pemphigus
(PNP). This disorder is a complication of
cancer

9. Clinical Manifestations
• oral lesions appearing as irregularly shaped
erosions that are painful, bleed easily, and heal
slowly.
• The skin bullae enlarge, rupture, and leave large,
painful eroded areas that are accompanied by
crusting and oozing

10. • A characteristic offensive odor emanates from
the bullae and the exuding serum.
• Nikolsky’s sign:
The Nikolsky sign is dislodgement of intact
superficial epidermis by a shearing force.

11. Complications
• Secondary bacterial infection
• Fluid and electrolyte imbalance
• Hypoalbuminemia

12. Management
The goals of therapy are
• To bring the disease under control as rapidly as
possible
• To prevent loss of serum and the development of
secondary infection
• To promote re-epithelization

13. • Corticosteroids are administered in high doses
to control the disease and keep the skin free of
blisters.
• In some cases, corticosteroid therapy must be
maintained for life.

14. • Immunosuppressive agents (eg, Azathioprine,
Cyclophosphamide) may be prescribed to help
control the disease and reduce the corticosteroid
dose.
• Plasmapheresis (ie, plasma exchange)
temporarily decreases the serum antibody level
and has been used for life-threatening cases.

15. NURSING DIAGNOSES
• Acute pain of skin and oral cavity related to blistering
and erosions
• Impaired skin integrity related to ruptured bullae and
denuded areas of the skin
• Anxiety and ineffective coping related to the
appearance of the skin and no hope of a cure
• Deficient knowledge about medications and side
effects

16. Nursing interventions
• Meticulous oral hygiene is important to keep the
oral mucosa clean and allow the epithelium to
regenerate.
• Frequent rinsing of the mouth is prescribed to
rid the mouth of debris and to soothe ulcerated
areas.
• The lips are kept moist with lip balm

17. • Cool wet dressings are protective and soothing.
• The patient with painful and extensive lesions
should be pre-medicated with analgesics before
skin care is initiated.
• Hypothermia is common, and measures to keep
the patient warm and comfortable are priority
nursing activities

18. • After the patient’s skin is bathed, it is dried
carefully and dusted liberally with non-irritating
powder, which enables the patient to move freely
in bed.

19. Reducing anxiety of the patient:
• The patient is encouraged to express freely anxieties,
discomfort, and feelings of hopelessness.
• Arranging for a family member or a close friend to
spend more time with the patient can be supportive.
• Referral for psychological counseling may assist the
patient in dealing with fears, anxiety, and depression.

20. Prevention of infection:
• The skin is cleaned to remove debris and dead
skin and to prevent infection
• The oral cavity is inspected daily, and any changes
are reported.
• Assess for signs and symptoms of local and
systemic infection.

21. • Antimicrobial agents are administered as
prescribed, and response to treatment is assessed.
• Health care personnel must perform effective hand
hygiene and wear gloves.
• In the hospitalized patient, environmental
contamination is reduced as much as possible.
• Protective isolation measures and standard
precautions are warranted.

22. • Blood component therapy may be prescribed
to maintain the blood volume, hemoglobin
level, and plasma protein concentration.
• Serum albumin, protein, hemoglobin, and
hematocrit values are monitored.

23. • The patient is encouraged to maintain adequate oral
fluid intake.
• Cool, non-irritating fluids are encouraged to
maintain hydration.
• Small, frequent meals or snacks of high-protein, high
calorie foods help maintain nutritional status.
• Parenteral nutrition is considered if the patient
cannot eat an adequate diet.