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Br J Dermatol. 2011 Sep;165(3):486-91. doi: 10.1111/j.1365-2133.2011.10401.x. Epub 2011 Jul 28.

The association between porphyria cutanea tarda and diabetes mellitus: analysis of a long-term follow-up cohort.

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1
Department of Dermatology, Hospital Clinic, IDIBAPS, University of Barcelona, 170 Villarroel, 08036 Barcelona, Spain. carlosmunozsantos@gmail.com

Abstract

BACKGROUND:

An association between porphyria cutanea tarda (PCT) and diabetes mellitus (DM) is widely reported, but the pathogenetic link remains unknown.

OBJECTIVES:

To investigate the natural history of DM in the setting of PCT and to determine which PCT features and risk factors may be associated with the development of DM.

METHODS:

This retrospective longitudinal study included 81 Spanish patients with PCT with at least 10 years of strict follow-up. Patients attended our Porphyria Unit for follow-up visits and the data were collected in the period 2004-2008. We classified patients into two groups: patients with glucose metabolism alterations (GMA: DM or impaired fasting glucose), and patients without. PCT features and PCT and DM risk factors were retrieved from clinical charts and compared between groups.

RESULTS:

We identified 33 patients (41%) with GMA, of whom 27 (82%) developed GMA a long time after the diagnosis of PCT (mean 12·7 years). In bivariate analysis, these patients had significantly higher mean serum ferritin at diagnosis (651 vs. 405 ng mL(-1); P = 0·005), a higher prevalence of persistently elevated serum ferritin (52% vs. 15%; P < 0·001 for trend) and a higher prevalence of family history of DM (48% vs. 19%; P = 0·004). In multivariate analysis, persistently elevated serum ferritin [odds ratio (OR) 10·66, 95% confidence interval (CI) 1·95-58·19; P = 0·006] and family history of DM (OR 4·82, 95% CI 1·34-17·33; P = 0·016) remained significantly associated with the presence of GMA.

CONCLUSIONS:

GMA are highly prevalent in patients with PCT and mostly develop a long time after the diagnosis of PCT. Persistent hyperferritinaemia seems to be a risk biomarker of GMA in patients with PCT, probably in the setting of chronic iron overload and hepatic inflammation. Strict long-term monitoring of glucose metabolism and serum ferritin may be advisable in the routine follow-up of patients with PCT.

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