Pseudoporphyria: discussion of etiologic agents

Abstract

Pseudoporphyria describes a photodistributed bullous disorder with negative urinary, fecal, and serum porphyrin studies. Although pseudoporphyria is thought to be extremely rare (less than 100 reported cases4-5), we propose that this entity is underreported. One author (KB) has seen four cases of pseudoporphyria in the past four years. We describe a patient with nonpruritic, nonpainful bulla on the dorsum of his hands. Biopsy revealed a split at the dermal-epidermal junction; laboratory tests and urinary porphyrin evaluation were negative.