Pseudoporphyria Treatment & Management

Updated: Jun 21, 2016
  • Author: Vineet Mishra, MD, FAAD; Chief Editor: Dirk M Elston, MD  more...
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Treatment

Medical Care

The primary treatment of pseudoporphyria is to discontinue the offending agent whenever possible. This may be facilitated by substituting certain medications (eg, tolmetin instead of naproxen for juvenile rheumatoid arthritis, nonthiazide agents for hypertension) for alternative agents that have not been associated with pseudoporphyria. [6, 12] Additionally, sun protection is used as both therapy and prophylaxis against recurrent eruptions, and patients should be adequately educated on this topic. [61] Patients should avoid direct sun exposure, wear sun-protective clothing, and apply titanium oxide‒ or zinc oxide‒based sunscreens, which are resistant to wavelengths that are known to induce porphyric eruptions (400-440 nm). [61] Resolution of the clinical findings may take many months, particularly in drug-induced pseudoporphyria.

In addition to discontinuation of causative agents, N-acetylcysteine (a glutathione precursor) has been reported to improve dialysis associated pseudoporphyria. [6, 64, 65] Proponents of this therapy have proposed that patients on hemodialysis have decreased levels of glutathione,4,5 (an antioxidant) and that reactive oxygen species may contribute to skin damage. [65] One case series involving two patients with pseudoporphyria demonstrated rapid healing of lesions in both patients after the introduction of N-acetylcysteine (with one patient receiving 200 mg four times daily and the other 600 mg twice daily) to treatment, which was otherwise unchanged. [65] It was also noted that discontinuation of the medication in one patient led to a recurrence of blistering. Further investigations evaluating the efficacy are needed to confirm results. However, oral N-acetylcysteine has few, mild adverse effects consisting of nausea, vomiting, and diarrhea and has been suggested to be a safe therapeutic option for pseudoporphyria complicating hemodialysis. [65]