Pemphigus is a blistering autoimmune disease that affects the skin and mucous membranes. pemphigus vulgaris It occurs when antibodies attack Desmoglein 3 (a protein that is present in the epidermal layer of skin).

1. PEMPHIGUS

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

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 Penicillin 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).

8. • Pemphigus foliaceus (PF) is the least severe of
the three varieties. Desmoglein 1, the protein that
is destroyed by the autoantibody.
• 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. 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

12. 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.

13. • 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.

14. NURSING INTERVENTIONS
• 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.
• Cool wet dressings are protective and soothing.

15. • 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
• 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.

16. 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

17. 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.
Antimicrobial agents are administered as prescribed, and response to
treatment is assessed.
• Health care personnel must perform effective hand hygiene and wear
gloves . environmental contamination is reduced as much as possible.
• Protective isolation measures and standard precautions are warranted.

18. • 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.
• 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.

19. COMPLICATIONS
• Secondary bacterial infection
• Fluid and electrolyte imbalance
• Hypoalbuminemia