A CROSS SECTIONAL AND OBSERVATIONAL STUDY OF DERMATOLOGY LIFE QUALITY INDEX SCORE IN GENODERMATOSES PATIENTS AT A TERTIARY CARE HOSPITAL.

Abstract

Although genodermatoses accounts for a small number of patients in dermatology practice, their diagnosis is of immense significance as most of these are life-long, multi- system disorders with limited treatment options. Some of them risk malignancy and account for premature deaths 1 . Common genodermatoses which we come across in practice belongs to the ichthyosis group, followed subsequently by epidermolysis bullosa, ectodermal dysplasias, albinism, cutis laxa, xeroderma pigmentosum and dyskeratosis congenita 3 .As these can cause cosmetic disfigurement, patients present to the dermatologist for their unusual appearance 2 . The rarity of the diseases and scant awareness causes major roadblocks in the management and research on patients with genodermatoses. Finlay and Khan have designed DLQI ( Dermatology Life Quality Index) to assess the social and psychological effect of dermatological conditions on the quality of life in 3 adults . As most genodermatoses lead to disfigurement and cause psychological distress, assessing the DLQI in them may provide some insight into patient’s psyche, foster patient-physician relationship thereby leading to better compliance and treatment out comes.