Background: Variegate porphyria (VP) is a rare disorder of haem biosynthesis. We report a novel association with hepatitis A infection.
Patient and methods: A 31-year-old man was diagnosed with acute hepatitis A infection. During recovery, he presented with abdominal pain and a photoaggravated blistering skin eruption.
Results: Urine porphyrin precursors were markedly raised with high coproporphyrin III isomer levels. Faecal protoporphyrin levels were markedly increased and a maximum plasma fluorescence emission at 629 nm was noted.
Discussion: Acute hepatitis A infection, and the associated metabolic stress exerted on the haem biosynthetic pathway, induced overt presentation of latent VP.
Learning points: There should be a high index of suspicion for an acute cutaneous porphyria when a photosensitive rash is accompanied by neurovisceral symptoms.Latent porphyria may be overtly manifested after appropriate triggers which stress the metabolic haem biosynthetic pathway. One such trigger demonstrated by this case presentation is acute hepatitis A infection.The diagnostic approach to the investigation of a suspected acute cutaneous porphyria is initially with light-protected samples for urinary porphyrin precursors and plasma for fluorescence scanning. These should be sampled ideally during symptomatic periods. Further specialist analysis with fractionation of urinary and faecal porphyrins is necessary to distinguish between the two different acute cutaneous porphyrias.
Keywords: Variegate porphyria; hepatitis A.
© EFIM 2020.