Pseudoporphyria

Porphyrias are metabolic disorders of heme synthesis. Partial enzymic deficiencies result in excessive accumulation and excretion of 5-aminolevulinic acid, porphobilinogen, and/or porphyrins. Porphyria cutanea tarda (PCT) is the most common of the porphyrias in North America and Europe. First described by Waldenström in 1937, this blistering disorder is caused by a deficiency of uroporphyrinogen decarboxylase, an enzyme in heme biosynthesis.1 Porphyrins accumulate in the liver, are transported in plasma, and are excessively excreted in the urine.

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