Evidence-based consensus guidelines for the diagnosis and management of erythropoietic protoporphyria and X-linked protoporphyria

Written By Anna Mann

Journal of the American Academy of Dermatology, Volume 89, Issue 6, 1227 - 1237

These guidelines were developed by a multidisciplinary panel through systematic review and consensus and outline the best practices for identifying, monitoring, and treating the protoporphyrias (EPP & XLP).

The guidelines include:

  • Diagnostic recommendations

  • Prevention of phototoxic reactions

  • Acute symptom relief, including nonpharmacologic measures

  • Detailed strategies for monitoring liver function, iron status, and vitamin D levels.

  • Special situations—such as surgery, pregnancy, and the safety of concurrent medications.

 

Authors

Amy K. Dickey, Hetanshi Naik, Siobán B. Keel, Cynthia Levy, Simon W. Beaven, Sarina B. Elmariah, Angelika L. Erwin, Robert J. Goddu, Karli Hedstrom, Rebecca K. Leaf, Mohamed Kazamel, Marshall Mazepa, Lisa Liang Philpotts, John Quigley, Haya Raef, MD, Sean R. Rudnick, Behnam Saberi, Manish Thapar, Jonathan Ungar, Bruce Wang and Manisha Balwani

Anna Mann
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Evidence-based consensus guidelines for the diagnosis and management of protoporphyria-related liver dysfunction in ePP & XLP