Editing Porphyria (section)

===Erythropoietic porphyria===
These are associated with accumulation of porphyrins in erythrocytes and are rare.

The pain, burning, swelling, and itching that occur in erythropoietic porphyrias (EP) generally require avoidance of bright sunlight. Most kinds of [[sunscreen]] are not effective, but SPF-rated long-sleeve shirts, hats, bandanas, and gloves can help. [[Chloroquine]] may be used to increase porphyrin secretion in some EPs.<ref name=Thadani/> [[Blood transfusion]] is occasionally used to suppress innate heme production.{{citation needed|date=November 2021}}

The rarest is congenital erythropoietic porphyria (CEP), otherwise known as [[Gunther's disease]]. The signs may present from birth and include severe photosensitivity, brown teeth that fluoresce in ultraviolet light due to deposition of Type 1 porphyrins, and later [[hypertrichosis]]. Hemolytic anemia usually develops. Pharmaceutical-grade [[beta carotene]] may be used in its treatment.<ref>Martin A Crook.2006. Clinical chemistry and Metabolic Medicine. seventh edition. Hodder Arnold. {{ISBN|0-340-90616-2}}</ref> A bone marrow transplant has also been successful in curing CEP in a few cases, although long-term results are not yet available.<ref name="pmid18186900">{{cite journal |vauthors=Faraci M, Morreale G, Boeri E, Lanino E, Dallorso S, Dini G, Scuderi F, Cohen A, Cappelli B | title = Unrelated HSCT in an adolescent affected by congenital erythropoietic porphyria | journal = Pediatr Transplant | volume = 12 | issue = 1 | pages = 117–120 | year = 2008 | pmid = 18186900 | doi = 10.1111/j.1399-3046.2007.00842.x | s2cid = 20789520 | doi-access = free }}</ref>

In December 2014, [[afamelanotide]] received authorization from the [[European Commission]] as a treatment for the prevention of [[phototoxicity]] in adult patients with EPP.<ref>{{cite web|title=Community register of medicinal products for human use|url=http://ec.europa.eu/health/documents/community-register/html/h969.htm#EndOfPage|website=ec.europa.eu|publisher=European Commission|access-date=24 December 2014|url-status=live|archive-url=https://web.archive.org/web/20141224180750/http://ec.europa.eu/health/documents/community-register/html/h969.htm#EndOfPage|archive-date=24 December 2014|df=dmy-all}}</ref> In a 2023 industry-funded phase 2 trial, dersimelagon, an orally administered, selective melanocortin 1 receptor agonist that increases levels of skin eumelanin, was reported to have increased the duration of symptom-free sunlight exposure and quality of life compared to placebo in patients with erythropoietic protoporphyria.<ref>{{Cite journal |last1=Balwani |first1=Manisha |last2=Bonkovsky |first2=Herbert L. |last3=Levy |first3=Cynthia |last4=Anderson |first4=Karl E. |last5=Bissell |first5=D. Montgomery |last6=Parker |first6=Charles |last7=Takahashi |first7=Fumihiro |last8=Desnick |first8=Robert J. |last9=Belongie |first9=Kirstine |date=2023-04-13 |title=Dersimelagon in Erythropoietic Protoporphyrias |url=http://www.nejm.org/doi/10.1056/NEJMoa2208754 |journal=New England Journal of Medicine |language=en |volume=388 |issue=15 |pages=1376–1385 |doi=10.1056/NEJMoa2208754 |pmid=37043653 |s2cid=258110676 |issn=0028-4793}}</ref>