title: Ascorbic acid deficiency in porphyria cutanea tarda.
abstract: Porphyria cutanea tarda (PCT), the most common form of porphyria, is manifested as skin photosensitivity caused by excess hepatic production of uroporphyrin and heptacarboxylporphyrin. In experimental animal models, ascorbic acid modulates chemically induced uroporphyrin accumulation. The purpose of this study was to determine whether ascorbic acid is decreased in the plasma of patients with PCT. Plasma was obtained after an overnight fast from 21 PCT patients, 16 of whom were infected with hepatitis C virus (HCV), and from a separate group of 9 patients with HCV infection but not PCT. Thirteen PCT patients were studied when they had active disease and 8 after treatment-induced remission. Plasma ascorbic acid was low (<23 micromol/L) in 11 (85%) of the 13 untreated PCT patients and deficient (<11 micromol/L) in 8 (62%). Two patients with normal ascorbic acid levels (45 and 62 micrommol/L) had consumed multivitamins. In 2 patients with deficient ascorbic acid, plasma levels returned to normal after phlebotomy treatment. Of the 8 patients studied during remission, 4 had normal ascorbic acid values and 4 were deficient (5 to 8 micromol/L). Plasma ascorbic acid values were normal for all patients who had HCV but no PCT. These data suggest that plasma ascorbic acid concentrations are commonly low in PCT, but this decrease is unrelated to HCV infection. Ascorbic acid deficiency may be one of the factors that contributes to the pathogenesis of PCT.
authors.affiliation: Veterans Administration Medical Center, White River Junction, Vermont 05009, USA.
authors.forename: P R
authors.initials: PR
authors.lastname: Sinclair
authors.forename: N
authors.initials: N
authors.lastname: Gorman
authors.forename: S I
authors.initials: SI
authors.lastname: Shedlofsky
authors.forename: C P
authors.initials: CP
authors.lastname: Honsinger
authors.forename: J F
authors.initials: JF
authors.lastname: Sinclair
authors.forename: M R
authors.initials: MR
authors.lastname: Karagas
authors.forename: K E
authors.initials: KE
authors.lastname: Anderson
mesh_terms: D000328:Adult; D000410:Alanine Transaminase / Q000097:blood; D001205:Ascorbic Acid / Q000097:blood; D001206:Ascorbic Acid Deficiency / Q000150:complications* / Q000821:virology; D001219:Aspartate Aminotransferases / Q000097:blood; D005260:Female; D005293:Ferritins / Q000097:blood; D006526:Hepatitis C / Q000097:blood / Q000150:complications; D006801:Humans; D008297:Male; D008875:Middle Aged; D010865:Pilot Projects; D017119:Porphyria Cutanea Tarda / Q000097:blood / Q000150:complications*; D014168:Transferrin / Q000378:metabolism
chemical_list: D014168:Transferrin; D005293:Ferritins; D001219:Aspartate Aminotransferases; D000410:Alanine Transaminase; D001205:Ascorbic Acid