Dextrose’s Impact on Serum Ferritin in Porphyria Cutanea Tarda: A Single-Case Insight

Porphyria cutanea tarda (PCT) is the most common type of porphyria, caused by a deficiency in the enzyme uroporphyrinogen decarboxylase (UROD).[1] This leads to the accumulation of porphyrins, which are photosensitizing compounds. It is the most common form of porphyria, with a prevalence of around 1 in 10,000 cases. It is caused by the deficiency of UROD. This enzyme is inhibited competitively by uroporphomethene formed due to oxidation of uroporphyrinogen produced by reactive oxygen species in the liver in the presence of iron.[2] Elevated serum ferritin levels are a common finding in PCT, and iron overload can worsen the condition.[3] Dextrose therapy, which promotes heme synthesis and reduces porphyrin production, has been suggested as a potential treatment. This report details the case of a patient with PCT treated with dextrose, monitoring the changes in serum ferritin levels. We are reporting a case of a 45-year-old male diagnosed with PCT 15 years ago, presenting with skin blistering, hyperpigmentation, and increased skin fragility. He also complained of pinkish discoloration of urine. Bullae would develop over hands, face, and forearm which would rupture on minor trauma and have healed with atrophic scarring and mottled type hypo and hyperpigmentation [Figure 1]. He also had hypertrichosis involving his face, limbs, and trunk. No significant history of alcohol consumption or Diabetes was present. He was diagnosed with a case of PCT based on a genetic study (observed stop gained c.424C>T (p.Arg142Ter) variant in UROD gene-heterozygous). The patient was taking low-dose hydroxychloroquin and antioxidants but he was not improving yet.Figure 1: Atrophic scarring and mottled pigmentation over face and handsRoutine blood investigations were normal including HIV, Hepatitis-B and C. There were elevated serum ferritin (605 ng/mL), increased urinary, fecal, and serum uroporphyrins and coproporphyrins [urine creatinine 0.47 g/L, uroporphyrin/creatinine 11482.0 mcg/g creatinine, heptaporphyrin/creatinine 7912.47 mcg/g creatinine, hexaporphyrin 727.09 mcg/g creatinine, pentaporphyrin/creatinine 1829.62 mcg/g creatinine, copro porphyrin total/creatinine 769.04 mcg/g creatinine, coproporphyrin I 49.14%, and Uro/Copro porphyrin 14.93]. The patient was started on intravenous dextrose therapy (50 g daily) aimed at reducing porphyrin production. Low-dose hydroxychloroquin (200 mg weekly) was continued. No other changes to diet or lifestyle were made during the treatment period. Liver function tests and serum ferritin levels were monitored monthly. At 2 weeks follow-up, the patient's serum ferritin level decreased to 410 ng/mL. Skin symptoms showed mild improvement. At 4 weeks follow-up serum ferritin further decreased to and normalized at 245 ng/mL. Blistering and skin fragility showed significant improvement [Figure 2].Figure 2: Post-treatment (week 4) showing improvement in cutaneous lesionsDextrose therapy in this patient with PCT was an effective adjuvant treatment with low-dose hydroxychloroquine in reducing serum ferritin levels and improving clinical symptoms. Dextrose therapy in acute porphyrias has shown a potential to reduce liver porphyrin synthesis. Since glucose can down-regulate the synthesis of heme precursors, intravenous dextrose or carbohydrate-rich diets can help control symptoms and prevent acute episodes.[4] In healthy individuals, glucose intake has been observed to increase circulating levels of hepcidin and subsequently lower serum iron levels. However, it remains unclear whether this increase in hepcidin originates primarily from liver cells (hepatocytes) or from insulin-producing pancreatic β-cells, which may also contribute to local hepcidin production.[5] This case report demonstrates that dextrose therapy can be an effective adjuvant treatment for PCT, particularly in reducing serum ferritin levels and improving clinical outcomes. Further studies with larger sample sizes are needed to confirm these findings and to establish dextrose therapy as a standard treatment for PCT. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.