Porphyria Cutanea Tarda in a Swedish Population: Risk Factors and Complications

Abstract

There are varying reports on the prevalence of risk factors in porphyria cutanea tarda (PCT). We reviewed 84 patients with PCT in a restricted uptake area in Gothenburg, Sweden and evaluated different potential risk factors for the disease and complications. Besides a thorough medical history, the patients were investigated with urinary porphyrin analyses, transferrin saturation, ferritin and liver tests. Subsamples of patients were tested for antibodies to hepatitis C virus (n = 68), haemochromatosis gene mutations (n = 58) and with the oral glucose tolerance test (n = 31). We found a prevalence of about 1 patient with PCT in 10 000 inhabitants. Nineteen (23%) patients reported heredity for PCT. Identified risk factors were alcohol abuse (38% of male patients), oestrogen treatment (55% of female patients), anti-hepatitis C virus positivity (29% of male patients), diabetes (17%) or impaired glucose tolerance (45% of tested patients) and haemochromatosis gene mutations (57% of tested patients). All patients positive for anti-hepatitis C virus belonged to the non-hereditary group. During follow-up we observed a high incidence of stroke, no case of hepatocellular carcinoma and a normal life expectancy.

5-aminolevulinic acid, a precursor of heme, reduces both fasting and postprandial glucose levels in mildly hyperglycemic subjects

“…The reduction in ALA-dehydratase activity reported in diabetic rats [8][9][10], and the high prevalence of diabetes or IGT in porphyria cutanea tarda [7], may indicate a connection between the heme biosynthetic pathway and diabetes. Heme deficiency may be a factor in neurodegenerative process [24,25], and it has been suggested that hyperinsulinemia and/or abnormal glucose tolerance are associated with a higher risk for Alzheimer's disease [26,27].…”

“…defective insulin signaling may promote mitochondrial dysfunction because the mitochondrial function is lower in patients with genetically defective insulin receptors than in healthy controls [6]. Porphyria cutanea tarda is caused by abnormalities in heme biosynthesis and often is accompanied by DM [7]. Moreover, the activity of ALA dehydratase is found to be reduced in a streptozotocin-induced diabetic rat model [8][9][10].…”

Smokers Report More Psoriasis, but Not Atopic Dermatitis or Hand Eczema: Results from a Norwegian Population Survey among Adults

“…Some cutaneous conditions are direct consequences of alcohol abuse, but alcohol seems to increase the risk of some skin diseases such as psoriasis, infectious eczematoid dermatitis and porhyria cutanea tarda [5, 22, 23]. As for psoriasis, studies have shown that alcohol-related mortality is increased among hospital-treated psoriasis patients [24].…”

Clinical and Laboratory Diagnosis of the Porphyrias

“…1 Diabetes and hepatic steatosis may contribute to oxidative stress and increase susceptibility for developing PCT. 40,197 Reported associations of this disease with systemic lupus erythematosis and other immunological disorders are unexplained. 198,199 …”