Pseudoporphyria

Abstract

Pseudoporphyria is the term used to describe a photodistributed bullous disorder with clinical and histologic features of porphyria cutanea tarda, but without accompanying biochemical porphyrin abnormalities. Medications, chronic renal failure/dialysis, excessive sun exposure and UVA radiation have all been reported to cause pseudoporphyria. Recognition, diagnosis, and appropriate management of pseudoporphyria are discussed.

Blisters, ulcers, crusts, and atrophic scars on the back of the hands and the extensor aspects of the forearms

“…Potential triggers of pseudoporphyria include numerous drugs, including tetracyclines, diuretics, dapsone, cyclosporine A, and retinoids. However, the most common drug known to cause pseudoporphyria is the NSAID naproxen, a derivative of propionic acid [ 4,5 ] . Among adults, females are predominantly affected [ 6 ] .…”

Pseudoporfiria induzida pela diálise tratada com N-acetilcisteína oral

“…N-acetylcysteine is also used in prophylactic therapy of contrast nephropathy as it prevents the increase of oxidative stress after contrast infusion. 1,6,10 We present a case of PP secondary to dialysis with good response to the use of oral N-acetylcysteine. The patient maintained the use of erythropetin and presented improvement of the lesions, suggesting that the condition was not medicamental.…”

“…There are reports of clinical control after the use of oral N-acetylcysteine. [1][2][3][4][5] It is presented here the case of a female patient, aged 32, who had been suffering from Goodpasture's syndrome for five years, evolving into chronic renal failure (anuria) and renal replacement therapy (peritoneal dialysis ) and placed on a transplant waiting list. The patients had been presenting recorrent bullae with hyaline content on the backs on the hands and feet for one year.…”

“…(Chart 1). 1 The exact pathophysiological mechanism of PP is not known. The formation of phototoxic metabolites in individuals geneticaly predisposed can be responsible for triggering bullous lesions.…”

“…[2][3][4] As for PP secondary to drugs, the suspension of these drugs leads to remission of the skin condition. 1 The treatment is based on avoiding sun exposure, topical steroids and also on avoiding potential medication involved. Case reports of prolonged use of N-acetylcysteine in such patients on hemodialysis have shown success.…”