Phemphigus

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 affect s 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.