Porphyria cutanea tarda in Scotland: underlying associations and treatment approaches
Issued Date
2024-01-01
Resource Type
ISSN
00119059
eISSN
13654632
Scopus ID
2-s2.0-85191148361
Journal Title
International Journal of Dermatology
Rights Holder(s)
SCOPUS
Bibliographic Citation
International Journal of Dermatology (2024)
Suggested Citation
Chaiyabutr C., Dawe R., Ibbotson S.H., Clarke T., McGuire V.A. Porphyria cutanea tarda in Scotland: underlying associations and treatment approaches. International Journal of Dermatology (2024). doi:10.1111/ijd.17205 Retrieved from: https://repository.li.mahidol.ac.th/handle/20.500.14594/98180
Title
Porphyria cutanea tarda in Scotland: underlying associations and treatment approaches
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Abstract
Background: Despite its rarity, porphyria cutanea tarda (PCT) is globally recognized as the most common form of cutaneous porphyria. This study aims to review the underlying associations and treatment of PCT in Scotland. Methods: We retrospectively reviewed data on 27 patients diagnosed with PCT between 1987 and 2022 at the Scottish Cutaneous Porphyria Service. Results: Males slightly predominated (66.7%). The mean ± standard deviation (SD) age at diagnosis was 55.6 ± 12.5 years. Common associated factors were heavy alcohol intake (88.5%), genetic hemochromatosis (72%), smoking (45.5%), and hepatitis C virus infection (16%). Most had multiple associated factors (70.4%). Patients with genetic hemochromatosis with the C282Y genotype exhibited higher median transferrin saturation (69.5 vs. 35, P = 0.004) and ferritin levels (observed in males only) (1175 vs. 339; P = 0.014) than those with the H636D genotype. Most (52%) received combination therapy of venesection and antimalarials, followed by venesection monotherapy (32%) and antimalarial monotherapy (16%). Overall, 95.2% achieved biochemical improvement. Median time to improvement was 7, 5, and 9 months with venesection, antimalarial, and combined treatments, respectively (P = 0.173). Biochemical remission was achieved in 50% of patients. Remission occurred in 2/4 of patients with antimalarial monotherapy (median time 19 months) and 9/13 patients with combined treatment (median time 26 months). Biochemical relapse was found in three patients, all of whom received combination therapy. Conclusion: Excess alcohol intake and genetic hemochromatosis were the most common underlying associations with PCT in our Scottish cohort. Treatment for PCT should be individualized, and long-term follow-up is needed to monitor for disease relapse.