Bullous Photosensitivity To Naproxen: “Pseudoporphyria”

Abstract

Pseudoporphyria is a photo-induced cutaneous bullous disease characterized by distinct clinical, histologic, and most recently, immunofluorescent features. By definition, results of porphyrin studies are normal in this disease. We describe here a woman with naproxeninduced pseudoporphyria, and we review previously reported cases of pseudoporphyria. The increasing frequency of pseudoporphyria is a result of the current popularity of nonsteroidal antiinflammatory drugs. Physicians need to be aware of this reversible skin disorder. Pseudoporphyria must be considered and an appropriate evaluation must be done when an individual who is taking nonsteroidal antiinflammatory drugs develops bullae and increased fragility of exposed skin.Porphyria cutanea tarda (PCT) is a blistering disorder caused by a deficiency of uroporphyrinogen decarboxylase, an enzyme in heme biosynthesis. Porphyrins accumulate in plasma and are excreted in the urine in great excess. Exposure to sunlight results in photosensitive lesions. PCT can be inherited or acquired. Treatment alternatives include phlebotomy and antimalarial agents ( 1 4 ) .

Pseudoporphyria and Nonsteroidal Antiinflammatory Agents in Children with Juvenile Idiopathic Arthritis

“…In contrast to true erythropoietic porphyria and porphyria cutanea tarda, milia formation, hypertrichosis, and waxy (sclerodermoid) skin changes do not occur and plasma porphyrin levels are not elevated. Pseudoporphyria has been linked to the use of certain drugs, for example, NSAIDs, (Table 1) and is a particular problem in patients receiving long‐term treatment for arthritis (1,2). It is also linked to ultraviolet A (UVA) exposure and tanning bed use (3), chronic renal failure, and hemodialysis (4), although in the latter there may be abnormalities of porphyrin metabolism, so strictly, the term pseudoporphyria is not applicable.…”

“…Some Drug Associations of PseudoporphyriaNSAIDS: propionic acid derivatives, for example, naproxen(1,2,12), oxaprozin, ketoprofen(12), nambumetone (13) Antibiotics: tetracycline(14), nalidixic acid(15,16),…”

Pseudoporphyria induced by voriconazole

“…1,4,5,11,12,33 DIF findings are similar to those in PCT, with granular deposits of IgG and C3 at the dermoepidermal junction and in the papillary dermal vasculature. [3][4][5][6][7]11,[15][16][17][18]33,36 Positive DIF is suggestive, but not required, for the diagnosis of pseudoporphyria. Indirect immunofluorescence is negative.…”

Nonsteroidal Antiinflammatory Drug-Induced Pseudoporphyria: A Case Series

“…Several reports stated that plasma or blood``porphyrin studies'' were within normal limits but did not give details of the assays per- formed. 10,19,20,31,37 Other reports had quanti®ed serum or plasma porphyrin fractions to make the diagnosis. 17,30,47 Plasma analysis for each of our patients was conducted by the Mayo Clinic Medical Laboratories for total plasma porphyrin levels rather than a fractionation for each of the porphyrin products.…”