Abstract

A CASE REPORT ON CHALLENGES IN TREATMENT OF PEMPHIGUS VULGARIS PATIENTS IN AN INDIAN TERTIARY CARE FACILITY; EMPHASIS ON DISEASE SIGNS AND SYMPTOMS AND CLASSICAL THERAPEUTIC MEASURES

Prestley Chia-Sani*, Dr. Blessy K. George and Dr. Shibi Mary Thomas

ABSTRACT

Pemphigus Vulgaris is the most common type of pemphigus and is an autoimmune dermatological disorder with an incidence rate of 0.1-0.5 per 100,000 people. Brought on by the attack of one‟s antibodies against desmosomes and hemidesmosomes on the body‟s epithelial cell layer, the identification of the onset of Pemphigus Vulgaris can be seen by the cutaneous lesions that appear on the individual's skin as well as buccal cavity amongst others. The global incidence rates vary from region to region, with studies carried out to pinpoint the most susceptible group which is noted by a high predominance level of Ashkenazi Jews. The diagnosis of this disease is based on positive signs shown by Nikolsky tests as well as the use of either a biopsy or immunofluorescences testing. The various treatment measures undergone to maintain the spread of the skin lesions have been greatly aided by the use of corticosteroids which aim to suppress the body‟s immune system from attacking itself. Primary therapy has been the use of systemic glucocorticoids by using a standard mode of treatment known as “Pulse Therapy”, which utilizes a combination of single-use administration of drugs such as dexamethasone, cyclophosphamide along with adjuvant drugs such as azathioprine, mycophenolate, dapsone, methotrexate, cyclosporine, and others. This case report describes the case of a 48-year-old male and a 58-year-old female with known cases of Pemphigus Vulgaris as well as the treatment measures they underwent.

Keywords: Pemphigus Vulgaris, Desmogleins, Nikolsky sign, Immunosuppressants, Pulse therapy, Cyclophosphamide, Dexamethasone.