P>Background Severe psoriasis is associated with excess mortality and increased risk of cardiovascular death. Population-based data evaluating cause-specific mortality in patients with psoriasis are limited. Objectives To describe cause-specific mortality in patients with severe psoriasis. Methods We performed a cohort study from 1987 to 2002 of patients >= 18 years using the General Practice Research Database. We compared patients with a psoriasis code and a history of systemic therapy consistent with severe psoriasis (n = 3603) with patients with no history of psoriasis (n =14 330). Age- and sex-adjusted Cox models were created for each of the leading causes of death defined by the Centers for Disease Control. Results Patients with severe psoriasis were at increased risk of death from cardiovascular disease [hazard ratio (HR) 1 center dot 57, 95% confidence interval (CI) 1 center dot 26-1 center dot 96], malignancies (HR 1 center dot 41, 95% CI 1 center dot 07-1 center dot 86), chronic lower respiratory disease (HR 2 center dot 08, 95% CI 1 center dot 24-3 center dot 48), diabetes (HR 2 center dot 86, 95% CI 1 center dot 08-7 center dot 59), dementia (HR 3 center dot 64, 95% CI 1 center dot 36-9 center dot 72), infection (HR 1 center dot 65, 95% CI 1 center dot 26-2 center dot 18), kidney disease (HR 4 center dot 37, 95% CI 2 center dot 24-8 center dot 53) and unknown/missing causes (HR 1 center dot 43, 95% CI 1 center dot 09-1 center dot 89). The absolute and excess risk of death was highest for cardiovascular disease (61 center dot 9 and 3 center dot 5 deaths per 1000 patient-years, respectively). Conclusions Severe psoriasis is associated with an increased risk of death from a variety of causes, with cardiovascular death being the most common aetiology. These patients were also at increased risk of death from causes not previously reported, such as infection, kidney disease and dementia. Additional studies are necessary to determine the degree to which excess causes of death are due to psoriasis, its treatments, associated behaviours, or other factors.