BACKGROUND As an aid in the diagnosis and management of porphyria we have developed a method to fractionate and quantify plasma porphyrins and have evaluated its use in various porphyrias. METHODS We used HPLC with fluorometric detection to measure plasma concentrations of uroporphyrin I and III, heptacarboxyl III, hexacarboxyl III, pentacarboxyl III, and coproporphyrin I and III. We studied ...

Pseudoporphyria is an uncommon bullous disease with similar clinical and histopathological findings to porphyria cutanea tarda, in the absence of detectable porphyrin elevation. We present a 34-year-old man with clinical and histological findings compatible with porphyria cutanea tarda, whose his urinary uroporphyrin was negative and we concluded that he was a case of pseudoporphyria. Concomita...

A 72-year-old female with end-stage renal disease due to chronic glomerulonephritis had been treated with conventional haemodialysis for 6 years. She was referred three months ago for management of blisters and cutaneous fragility on the dorsa of both hands (Figure 1a and b). The patient denied extensive sun exposure, sun beds, or use of medications other than calcium carbonate, vitamin B compl...