Pemphigus

seminar

1. Pemphigus R.Anusha Pharm D V Year Roll No:170514882007

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

3. 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 patients . Usually occurs in men and women in middle and late adult-hood. May be associated with Penicillin's and Captopril and with Myasthenia Gravis .

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

5. T ypes There are three types of pemphigus which vary in severity: Pemphigus vulgaris Pemphigus foliaceus Paraneoplastic pemphigus.

6. Pemphigus vulgaris 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). 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.

7. Pemphigus foliaceus The least severe of the three types is Pemphigus foliaceus(PF) . Desmoglein-1 , is the protein that is destroyed by the autoantibody. It doesn’t cause blisters in the mouth. The blisters first appear on the face and scalp. Blisters then appear on the chest and back. The blisters are usually itchy and painless.

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

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. A characteristic offensive odour emanates from the bullae and the exuding serum. NIKOLSKY’S SIGN: The Nikolsky sign is dislodgement of intact superficial epidermis by a shearing force.

10. Diagnostic test Direct Immunofluorescence: A biopsy sample will be examined by a technique known as direct immunofluorescence to demonstrate the presence of pemphigus vulgaris autoantibodies in the skin. Indirect Immunofluorescence: Pemphigus autoantibodies can be measured in the blood.

11. MANAGEMENT THE GOALS OF THE THERAPY INCLUDE: 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. Corticosteroids are effective and work quicker than most other treatments. The corticosteroids used are synthetic versions of a natural hormone produced in smaller quantities by the adrenal gland and work by suppressing the immune system. High doses of corticosteroids, usually prednisolone, are given to bring pemphigus under control. The dose is then reduced slowly to minimize side effects.

13. Steroid-sparing drugs/Immunosuppressive agents . corticosteroids have many side effects when taken long-term at high doses. For this reason, other drugs are prescribed to allow the dose of steroid to be minimized. These are known as steroid-sparing drugs. They include the following: Immunosuppressive drugs: azathioprine, cyclophosphamide, mycophenolate mofetil, methotrexate and rituximab.

14. Additional drugs or treatments that may be used include intravenous immunoglobulins, tetracyclines (minocycline or doxycycline) combined with nicotinamide, dapsone. P l a s m a p h e r e s i s ( i.e , plasma exchange) temporarily decreases the serum antibody level and has been used for life-threatening cases. This treatment involves removing plasma from the blood. Plasmapheresis and intravenous immunoglobulin therapy may be considered if other treatments are not effective. They may be used in combination with steroid tablets.

15. REFERENCE: AMERICAN ACADEMY OF DERMATOLOGY British Association of Dermatologists guidelines on the management of Pemphigus 2017.