SA Holme, AV Anstey, NM Badminton*, GH Elder*
Departments of Dermatology and *Medical Biochemistry and Immunology, University of Wales, School of Medicine, Heath Park, Cardiff. CF14 4XN
Skin conditions may profoundly affect quality of life (QOL) through disruption of relationships, and interference with sport, leisure and work. Despite advances in understanding the molecular genetics and treatment of erythropoietic protoporphyria (EPP), little has done to assess the psychosocial consequences of this condition.1 A number of specifically-designed tools have been developed to measure health-related QOL for adults and children with skin disease. The aim of this study was to assess quality of life parameters in a large cohort of EPP patients in the UK. Two validated dermatology-specific quality of life measures were used: dermatology life quality index (DLQI)2 and the children’s dermatology life quality index (CDLQI) 3. Each index consists of ten questions each scored up to 3, giving a maximum possible score of 30. 223 EPP patients (114 females, 109 males) were recruited, and seen by the study investigator over a six-month period. 176 DLQIs and 44 completed CDLQIs were obtained. The mean total DLQI score was 13.95 (SD +/- 6.715, range 0-29). The highest scoring (highest disability) DLQI questions related to clothing, social/leisure activities and sport. The mean total CDLQI score was 13.02 (SD +/- 4.027, range 5-21). The highest CDLQI questions related to social/leisure activities, skin symptoms and sleep. These mean scores are in the upper range when compared to mean scores for other dermatological conditions such as acne (4.3-17.7), eczema (4.4-21.4), psoriasis (1.7-18.2), rosacea (6.3-7.8) and normal populations (0.3-0.5)4.
In conclusion, this is the first large-scale research project to have formally assessed quality of life parameters in EPP. Results obtained in this study show that EPP ranks as one of the most disabling of skin conditions, despite the relative paucity of visible signs.
References
Millward LM, Kelly P, Deacon A et al Self-related psychosocial consequences and quality of life in the acute porphyrias. J Inherit Metab Dis 2001; 24: 733-747.
Finlay AY, Khan GK. Dermatology life quality index (DLQI): a simple practical measure for routine clinical use. Clin Exp Dermatol 1994; 19: 210-6.
Holme SA, Man I, Sharpe JL et al. The children’s dermatology life quality index: validation of the cartoon version. Br J Dermatol 2003; 148 (2): 285-90.
Lewis V, Finlay AY. 10 years experience of the dermatology life quality index (DLQI). J Invest Dermatol Symp Proc 2004; 9: 169-180.