An Evaluation of Pharmacological Management of Pemphigus Vulgaris and its Impact on Quality of Life

Abstract

Pemphigus vulgaris is a rare autoimmune blistering disease of skin and mucous membranes. The Worldwide incidence of Pemphigus vulgaris is 0.5 to 3.2 cases per one lakh populations/year. 1 In India, incidence of Pemphigus vulgaris is 4.4 cases per million populations/year. 2 Patients of Pemphigus vulgaris are presented with flaccid, thin walled vesicles and/ or bullae which are commonly rupture to leave an area of erosion and ulceration. Involvement of various mucosal surfaces such as oral, ocular, nasal, pharyngeal, laryngeal, upper respiratory and anogenital mucous membrane is present in Pemphigus vulgaris. Majority of patients with Pemphigus vulgaris develop painful erosions of the oral and skin lesion due to involvement of peripheral nerves. 3 The choice of therapy depends on severity of the disease, efficacy, safety, availability and cost of drugs. 4 Corticosteroid are preferred therapy for Pemphigus vulgaris, azathioprine is used for its corticosteroid-sparing effect and it is first line adjuvant immunosuppressant agent. 5 Rituximab, an anti-CD20 monoclonal antibody is indicated for moderate to severe refractory cases, contraindication or severe ADR to systemic corticosteroids and immunosupressants. 6 High-dose Intravenous immunoglobulin G (IVIG) and plasmapheresis can also be indicated in resistant cases. 5 Disease symptoms e.g. painful lesions, disease severity and complications due to treatment e.g. ADR may adversely affect the quality of life of patients. 7 Therefore, quality of life measures can be used to guide and evaluate treatment interventions. During extensive literature search, we observed studies reported from different countries about efficacy (i.e. Pemphigus area and activity score, Pemphigus disease area index), evaluation of quality of life (i.e. skindex-29, short form-36, autoimmune Bullous Disease Quality of Life Questionnaire) and correlation of efficacy and QOL in patients of Pemphigus vulgaris. 8-10 We did not find any Indian study showing efficacy and correlation of efficacy with QOL over a period of time. Hence, this study was carried out to study the use and effect of the drugs as well as its impact on quality of life in patient suffering from Pemphigus vulgaris. MATERIALS AND METHODS Permissions to conduct the study were obtained from the Institutional Ethics committee (IEC No-208/15) and the Head of Department of Dermatology and Venereology. All newly diagnosed patients of Pemphigus vulgaris by Dermatologist, between 18 to 65 years of either gender, who are willing to participate in the study and gave written informed consent were enrolled. Patients who were suffering from severe or uncontrolled cases of hypertension, diabetes mellitus, hepatic, renal, pulmonary, hematologic disorders and pregnant as well as lactating women were excluded. This was an observational, prospective, single center study carried out at department of skin and venereal disease of a tertiary care teaching hospital over a period of 24 months from November 2015 to November 2017. The baseline data like demograp...