Experience in management of porphyria cutanea tarda in a tertiary referral Brazilian hospital from 2002 to 2017.

Abstract

Porphyria cutanea tarda (PCT) is the most common porphyria worldwide. The known acquired precipitating factors that induce PCT include alcoholism, hepatitis C virus infection, human immunodeficiency virus infection, and estrogen intake. Hereditary hemochromatosis is considered an inherited risk factor. The aim of this study was to describe and analyze precipitating factors and family history, with emphasis on PCT management. A retrospective study of 87 patients with PCT was conducted between January 2002 and December 2017. A male predominance of 1.8:1 was found. The median age at diagnosis was 49years (range 18-71). Family history of PCT was observed in 19.5% of patients. Two or more acquired precipitating factors were present in 42.5%. Patients were treated with antimalarial monotherapy (72.4%), antimalarial combined with phlebotomy (22.9%), and only with phlebotomy (4.6%). Acquired precipitating factors and inherited factors were not associated with treatment group. There was a difference in 24h-UP normalization rate between treatment groups; combined therapy takes longer than antimalarial monotherapy, 38months versus 15months, respectively (CI 95%, 6.5-63.5 vs. 12.9-17) (log-rank test, P=0.004). Precipitating factors did not seem to be associated with treatment choice; however, all acquired and inherited precipitating factors should be investigated, and the choice between phlebotomy and/or antimalarials should be individualized. All dermatologists treating PCT patients should observe transferrin saturation and ferritin levels to search for underlying hereditary hemochromatosis.