PEMPHIGUS VULGARIS [PV]

Aswathy K S, 1^st batch Pharm D.

Pemphigus is a chronic autoimmune mucocutaneous disease, with blister formation. The annual incidence of pemphigus reported is 1 to 5 per million populations per year.Pemphigus Vulgaris is a chronic autoimmune mucocutaneous disease. The disease primarily exhibit as intraoral lesions and then advance to other mucus membranes and skin.^[1] In PV, IgG autoantibodies are directed against a group of trans membrane adhesion proteins located in desmosomes and named desmogleins (Dsg), more specifically their subtypes 1 and 3 (cutaneo-mucous form) and 3 (mucous form), which leads to acantholysis in the suprabasalspinouslayer.^[2] Aetiology of pemphigus vulgaris is uncertain.^[3] Diagnosis can be conducted by physical examination of the skin blisters. Main indicator of the condition is positive Nikolsky’s sign in which the skin shears off easily when the surface is wiped sideways with a cotton swab or a finger.  Immunofluorescence or ELISA tests, to measure the level of the antibody that causes PV (the PV auto-antibody) in the bloodstream and the biopsy tests are the commonly used diagnostic methods. ^[2]

The main treatment goal is to reduce inflammatory responses, autoantibody production and thus to prevent disease progression.Untreated  Pemphigus can be fatal due to overwhelming infection of the sores. The main treatment options are Steroids, Immunosuppressant’s, Topical treatments, Plasmapheresis, Intravenous Immunoglobulin, and Monoclonal Antibodies etc. ^[2,4]

Management mainly comprises of corticosteroid with or without adjuvant drugs.  The side effects of cortico-steroids may require the use of so-called steroid-sparing or adjuvant drugs. Steroids reduce redness and soreness (inflammation) and suppress the immune system. A high intravenous dose is usually given initially. Adjuvant drugs are commonly used in combination with the aim of increasing efficacy and of having a steroid sparing action, thereby allowing reduced maintenance corticosteroid doses and reduced corticosteroid side effects. Patients with mild disease are treated with initial prednisolone doses of 40 to 60 mg per day and in more severe cases, 60 to 100 mg per day. If there is no response within 5 to 7 days, the dose should be increased in 50 to 100% increment until there is disease control. Immunosuppressant medicine works by suppressing the immune system. The oral lesion of PV may respond partially to topical corticosteroids (creams, pastes) but some form of systemic immunosuppressant’s is needed to control the level of circulating auto antibodies. Azathioprine is a commonly prescribed adjuvant drug in PV. Examples of immunosuppressant medicines which are used for PV are cyclophosphamide, azathioprine, ciclosporin, methotrexate or mycophenolatemofetil. Immunosuppressants usually take longer to work than steroids (about 4-6 weeks). Azathioprine doses of 1 to 3 mg/kg have been used but ideally should be titrated according to the individual activity of thiopurinemethyltransferase.Oral cyclophosphamide could be considered as an alternative to azathioprine. Mycophenolatemofetil( 2 to 2.5 gm in 2 divided doses) is a relatively new agent in PV therapy. Antibiotics, Antivirals, and Antifungals are used to prevent other infections. Steroid creams, Mouthwash with antiseptic and local anaesthetic, Wound care and dressings are mainly used for the wound management. Plasmapheresis, intravenous immunoglobulin and rituximab (a monoclonal antibody) can be used in cases if PV does not respond to high doses of steroids.Plasmapheresis is a process in which antibody-containing plasma is removed from the blood and replaced with intravenous fluids or donated plasma. Plasmapheresis may be used along with systemic medications to reduce the amount of antibodies in the blood. IVIG doses of 1.2 to 2 gm/kg divided over 3 to 5 days infused every 2 to 4 weeks for 1 to 34 cycles.

REFERENCES

1.      SandhyaTamgadge, AvinashTamgadge, Daivat M. Bhatt, SudhirBhalerao, Treville Pereira. Pemphigus Vulgaris.CCD. 2011: 2(2); 136-137.

2.      Shams UL Nisa, SC SelvaMuthukumar, NaliniAswath, BaluKarthika. Pemphigus Vulgaris: A Case report with review of literature. JIAOMR. 2013: 25(1); 55-58.

3.      Robinson NA,Yeo JF, Lee YS, Aw DC. Oral pemphigus vulgaris: A case  report and review of literature.AnnAcad Med Singapore 2004; 33(4 suppl);63-68

4.      Mohsen Masjedi, Ali Asilian, ZabihollahShahmoradi, ParvinRajabiDehnavi, BaharehAbtahiNaeini., Successful Treatment of Pemphigus Vulgaris With the Extensive Mucocutaneous Lesions in an Elderly Patient. Iran Red Crescent Med J. 2014; 16(6): 1-4.

5.      Baroni A, Lanza A, Cirillo N, Brunetti G, Ruocco E, Ruocco V. Vesicular and bullous disorders: pemphigus. DermatolClin. 2007; 25(4):597-603.