119:
65:
37:
195:; the remainder undergoes transformation into heme. Some protoporphyrin in bile is returned to the liver as a consequence of the enterohepatic circulation; the remaining protoporphyrin in the intestine undergoes fecal excretion. Protoporphyrin is insoluble and hence unavailable for renal excretion. In EPP, subnormal biotransformation of protoporphyrin into heme results in accumulation of protoporphyrin in hepatocytes.
154:
phototoxic reactions in 25% of patients who were unprotected by surgical light filters. The same study noted a 69% recurrence of the disease in the grafted organ. Five UK liver transplants for EPP have been identified between 1987 and 2009. Frequent liver testing is recommended in EPP patients where no effective therapy has been identified to manage liver failure to date.
131:
sexual development and maintenance. More severe EPP can manifest in infancy. EPP can be triggered through exposure to sun even though the patient is behind glass. Even the UV emissions from arc welding with the use of full protective mask have been known to trigger EPP. EPP can also manifest between the ages of 3 and 6.
182:. EPP exhibits both recessive and dominant patterns of inheritance and a high degree of allelic heterogeneity with incomplete penetrance. Most heterozygotes are asymptomatic. Symptoms do not occur unless FECH activity is less than 30% of normal, but such low levels are not present in a majority of patients.
190:
Cells which synthesize heme are predominantly erythroblasts/reticulocytes in the bone marrow (80%) and hepatocytes (20%). Deficiency of FECH results in increased release of protoporphyrin, which binds to albumin in plasma and subsequently undergoes hepatic extraction. Normally, most protoporphyrin in
130:
EPP usually first presents in childhood, and most often affects the face and the upper surfaces of the arms, hands, and feet and the exposed surfaces of the legs. Most patients, if the EPP is not as severe, manifest symptoms with onset of puberty when the male and female hormone levels elevate during
126:
EPP usually presents in childhood with the most common mode of presentation as acute photosensitivity of the skin. It affects areas exposed to the sun and tends to be intractable. A few minutes of exposure to the sun induces pruritus, erythema, swelling and pain. Longer periods of exposure may induce
407:
Although erythropoietic protoporphyria symptoms may be temporarily suppressed with cold temperatures, patients have found that this method may extend, or even intensify pain and discomfort. However, home treatments that increase blood flow to the affected areas, such as immersion in warm water with
309:
that is administered to promote biliary secretion of protoporphyrin. Results of its use in EPP are controversial. However, it is known to alter the composition of bile, to protect hepatocytes from the cytotoxic effect of hydrophobic bile acids, and to stimulate biliary secretion by several distinct
265:
can also be very helpful, although clothing with UPF values are only rated based on their UV protection (up to 400 nm) and not on their protection from the visible spectrum. Some sun protective clothing manufacturers use zinc oxide in their fabrics, such as
Coolibar's ZnO Suntect line, which
149:
Protoporphyrin accumulates to toxic levels in the liver in 5–20% of EPP patients, leading to liver failure. The spectrum of hepatobiliary disease associated with EPP is wide. It includes cholelithiasis, mild parenchymal liver disease, progressive hepatocellular disease and end-stage liver disease.
420:
has been estimated somewhere between 1 in 75,000 and 1 in 200,000 however it has been noted that the prevalence of EPP may be increasing due to a better understanding of the disease and improved diagnosis. An estimated 5,000–10,000 individuals worldwide have EPP. EPP is considered the most common
249:
can be a good indicator of what light is most detrimental, as the higher the color temperature, the more violet light (380–450 nm) is emitted. Incandescent and LED lighting in the soft white range (2700–3000 K) produce the least problematic light. Additionally, selecting lower wattage
215:
EPP is generally suspected by the presence of acute photosensitivity of the skin and can be confirmed by detection of a plasmatic fluorescence peak at 634 nm. It is also useful to find increased levels of protoporphyrin in feces and the demonstration of an excess of free protoporphyrin in
153:
A lack of diagnostic markers for liver failure makes it difficult to predict which patients may experience liver failure, and the mechanism of liver failure is poorly understood. A retrospective
European study identified 31 EPP patients receiving a liver transplant between 1983 and 2008, with
244:
There is no cure for this disorder; however, symptoms can usually be managed by limiting exposure to daytime sun and some types of artificial lighting. Most types of artificial lighting emit light in the problematic wavelengths, with fluorescent lighting being the worst offender.
198:
Since FECH deficiency is associated with increased concentrations of protoporphyrin in erythrocytes, plasma, skin and liver, retention of protoporphyrin in skin predisposes to acute photosensitivity. As a result of absorption of ultraviolet and visible light (peak sensitivity at
408:
quick drying or warm dry compresses, may increase the rate of pain relief. This has been noted as particularly effective in the hands, forearms, and face, as areas of decreased blood flow may be exposed to the accumulation of protoporphyrins for an extended period.
203:, with lesser peaks between 500–625 nm) by protoporphyrin in plasma and erythrocytes when blood circulates through the dermal vessels, free radicals are formed, erythrocytes become unstable and injury to the skin is induced.
226:
Liver biopsy confirms hepatic disease in EPP by the presence of protoporphyrin deposits in the hepatocytes that can be observed as a brown pigment within the biliary canaliculi and the portal macrophages. Macroscopically, the
206:
A significant increase in the hepatobiliary excretion of protoporphyrin can damage the liver through both cholestatic phenomena and oxidative stress—predisposing to hepatobiliary disease of varying degrees of severity.
231:
liver can have a black color due to protoporphyrin deposits. Using polarized light the characteristic
Maltese cross shape of birefringent crystalline pigment deposits is found. The examination of liver tissue under a
1259:
1921:
1901:
1441:
McCullough AJ, Barron D, Mullen KD, Petrelli M, Park MC, Mukhtar H, Bickers DR (January 1988). "Fecal protoporphyrin excretion in erythropoietic protoporphyria: effect of cholestyramine and bile acid feeding".
332:
which reduces circulating levels of protoporphyrin by binding to protoporphyrin in the intestine and, hence, interrupting the enterohepatic circulation. It is usually used in combination with other treatment
286:, developed by Australian-based Clinuvel Pharmaceuticals, was approved in Europe in December 2014 and in the United States in October 2019 for treatment or prevention of phototoxicity in adults with EPP.
339:, like cholestyramine, binds to protoporphyrin in the intestine and prevents its absorption. It is cheap and readily available. It seems to be effective in reducing circulating protoporphyrin levels.
162:
EPP photosensitivity symptoms are reported to lessen in some female patients during pregnancy and menstruation, although this phenomenon is not consistent, and the mechanism is not understood.
316:
appears to reduce excess protoporphyrin production in the bone marrow. It has been administered to patients with EPP (3–4 mg/kg iv) who develop a crisis after liver transplantation.
1406:
Tung BY, Farrell FJ, McCashland TM, Gish RG, Bacon BR, Keeffe EB, Kowdley KV (September 1999). "Long-term follow-up after liver transplantation in patients with hepatic iron overload".
909:
Kong XF, Ye J, Gao DY, Gong QM, Zhang DH, Lu ZM, et al. (February 2008). "Identification of a ferrochelatase mutation in a
Chinese family with erythropoietic protoporphyria".
1559:
Minder EI, Schneider-Yin X, Steurer J, Bachmann LM (February 2009). "A systematic review of treatment options for dermal photosensitivity in erythropoietic protoporphyria".
1251:
2257:
723:
1833:
Magnus IA, Jarrett A, Prankerd TA, Rimington C (August 1961). "Erythropoietic protoporphyria. A new porphyria syndrome with solar urticaria due to protoporphyrinaemia".
134:
Prolonged exposure to the sun can lead to edema of the hands, face, and feet, rarely with blistering and petechiae. Skin thickening can sometimes occur over time.
223:
mutation on one allele or aminolevulinic acid synthase 2 gain-of-function mutation in selected family members may be useful, especially in genetic counseling.
170:
Most cases of EPP are results of inborn errors of metabolism but the metabolic defect in some patients may be acquired. Mutation of the gene that encodes for
1273:
Pirlich M, Lochs H, Schmidt HH (December 2001). "Liver cirrhosis in erythropoietic protoporphyria: improvement of liver function with ursodeoxycholic acid".
2084:
269:
Window films which block UV and visible light up to 450 nm can provide relief from symptoms if applied to the patient's automobile and home windows.
2032:
127:
second degree burns. After repetitive exposure, patients may present with lichenification, hypopigmentation, hyperpigmentation and scarring of the skin.
399:
has been undergoing trials in
Australia since 2022 with some success in allowing participants to spend more time in full sunlight without ill effects.
600:"Erythropoietic protoporphyria: time to prodrome, the warning signal to exit sun exposure without pain-a patient-reported outcome efficacy measure"
381:(a gram-positive soil probiotic) produces ferrochelatase, which may be able to convert some of the protoporphyrin in the intestine into heme.
174:
in the long arm of chromosome 18 is found in majority of the cases. Ferrochelatase (FECH) catalyzes the insertion of ferrous iron into the
749:
Spelt JM, de Rooij FW, Wilson JH, Zandbergen AA (December 2010). "Vitamin D deficiency in patients with erythropoietic protoporphyria".
860:
272:
Blue blocking screen protectors can help provide relief from symptoms caused by televisions, phones, tablets and computer screens.
816:
Wahlin S, Marschall HU, Fischler B (June 2013). "Maternal and fetal outcome in
Swedish women with erythropoietic protoporphyria".
275:
EPP is considered one of the least severe of the porphyrias. Unless there is liver failure, it is not a life-threatening disease.
2025:
1649:
434:
1665:
Elder G, Harper P, Badminton M, Sandberg S, Deybach JC (September 2013). "The incidence of inherited porphyrias in Europe".
1201:
236:
reveals a red fluorescence due to protoporphyrin. Liver biopsy is not helpful for estimation of prognosis of liver disease.
1936:
2018:
322:
can also decrease the levels of protoporphyrin in plasma, however its use in treating acute episodes is controversial.
1877:
598:
Wensink D, Langendonk JG, Overbey JR, Balwani M, Van
Broekhoven EJ, Wagenmakers MA, et al. (September 2021).
555:
Seager MJ, Whatley SD, Anstey AV, Millard TP (January 2014). "X-linked dominant protoporphyria: a new porphyria".
1177:
429:
Erythropoietic protoporphyria was first described in 1953 by
Kosenow and Treibs and completed in 1960 by Magnus
2118:
2167:
2089:
792:
456:
110:
A clinically similar form of porphyria, known as X-Linked dominant protoporphyria, was identified in 2008.
1998:
652:
Tsuboi H, Yonemoto K, Katsuoka K (November 2007). "Erythropoietic protoporphyria with eye complications".
476:
2202:
2197:
1751:"Erythropoietic protoporphyria in Sweden: demographic, clinical, biochemical and genetic characteristics"
343:
1225:
2222:
1318:"Ursodeoxycholic acid in cholestatic liver disease: mechanisms of action and therapeutic use revisited"
2138:
695:
Poh-Fitzpatrick MB (June 1986). "Molecular and cellular mechanisms of porphyrin photosensitization".
1252:"FDA approves first treatment to increase pain-free light exposure in patients with a rare disorder"
64:
2059:
1947:
261:
which provides uniform protection between 290–400 nm and some protection up to 700 nm).
2114:
1872:
451:
387:, though a recent meta analysis of carotene treatment has called its effectiveness into question.
262:
2262:
91:, which varies in severity and can be very painful. It arises from a deficiency in the enzyme
724:"Erythropoietic Protoporphyria and X-Linked Protoporphyria - Hormonal and Metabolic Disorders"
358:, parenteral iron and transfusion of erythrocytes are alternative plans for treatment of EEP.
2192:
446:
347:
1520:"Purification and characterisation of a water-soluble ferrochelatase from Bacillus subtilis"
302:
1130:"Liver disease in erythropoietic protoporphyria: insights and implications for management"
8:
2252:
2148:
1925:
421:
form of porphyria in children. The prevalence in Sweden has been published as 1:180,000.
118:
1815:
1726:
1709:
1690:
1536:
1519:
1347:
1298:
1154:
1129:
1070:
1043:
1016:
989:
891:
841:
774:
677:
629:
580:
532:
505:
1930:
1846:
1106:
2055:
1958:
1850:
1807:
1772:
1767:
1750:
1731:
1682:
1645:
1568:
1541:
1500:
1459:
1455:
1423:
1388:
1383:
1366:
1339:
1290:
1286:
1159:
1110:
1075:
1021:
970:
926:
883:
833:
766:
704:
669:
665:
633:
621:
572:
537:
377:
246:
175:
49:
1819:
1694:
1351:
1302:
895:
845:
778:
681:
504:
Casanova-González MJ, Trapero-Marugán M, Jones EA, Moreno-Otero R (September 2010).
2143:
1987:
1842:
1799:
1762:
1721:
1674:
1531:
1490:
1451:
1415:
1378:
1329:
1282:
1149:
1141:
1102:
1065:
1055:
1011:
1001:
960:
918:
875:
859:
Blagojevic D, Schenk T, Haas O, Zierhofer B, Konnaris C, Trautinger F (July 2010).
825:
758:
661:
611:
584:
564:
527:
517:
336:
2109:
1952:
965:
948:
503:
355:
1614:
1587:
2227:
1006:
922:
616:
599:
351:
325:
319:
171:
96:
92:
1963:
1910:
1678:
949:"Erythropoiesis and iron metabolism in dominant erythropoietic protoporphyria"
879:
762:
36:
2246:
522:
384:
283:
54:
2010:
1334:
1317:
1145:
253:
Since the photosensitivity results from light in the visible spectrum, most
1854:
1811:
1776:
1735:
1686:
1572:
1504:
1427:
1343:
1294:
1163:
1114:
1079:
1060:
974:
930:
887:
837:
770:
673:
625:
576:
541:
1790:
Kosenow W, Treibs A (1953). "LichtĂĽberempfindlichkeit und
Porphyrinämie".
1545:
1495:
1478:
1463:
1392:
1365:
Potter C, Tolaymat N, Bobo R, Sharp H, Rank J, Bloomer J (November 1996).
1025:
708:
141:. One study has noted that EPP patients suffer from vitamin D deficiency.
1479:"Liver failure in protoporphyria: long-term treatment with oral charcoal"
1419:
100:
73:
58:
1893:
947:
Holme SA, Worwood M, Anstey AV, Elder GH, Badminton MN (December 2007).
1803:
793:"Erythropoietic Protoporphyria (EPP) and X-Linked Protoporphyria (XLP)"
417:
396:
258:
233:
138:
829:
568:
2172:
2064:
2050:
1982:
306:
295:
254:
228:
200:
88:
1710:"Erythropoietic protoporphyria: a case report and literature review"
1993:
506:"Liver disease and erythropoietic protoporphyria: a concise review"
107:. The severity varies significantly from individual to individual.
1558:
313:
1941:
1905:
1707:
1367:"Hematin therapy in children with protoporphyric liver disease"
597:
373:
is an oral heme supplement which may work similarly to
Hematin.
1832:
1708:
Michaels BD, Del Rosso JQ, Mobini N, Michaels JR (July 2010).
1440:
858:
748:
370:
329:
104:
1664:
2041:
1914:
192:
179:
554:
1405:
416:
Case reports suggest that EPP is prevalent globally. The
1748:
1178:"Zinc Oxide - Physical UVA+UVB sunscreen/sunblock agent"
499:
497:
1364:
946:
815:
651:
137:
People with EPP are also at increased risk to develop
1749:
Wahlin S, Floderus Y, StĂĄl P, Harper P (March 2011).
494:
1883:
2258:
Skin conditions resulting from errors in metabolism
2110:
Gunther disease/congenital erythropoietic porphyria
1371:
Journal of Pediatric Gastroenterology and Nutrition
1272:
942:
940:
257:are of little use (with the exception of non-nano
1714:The Journal of Clinical and Aesthetic Dermatology
1517:
1315:
2244:
721:
647:
645:
643:
1639:
1092:
1041:
937:
694:
1476:
250:bulbs can reduce the overall output of light.
2040:
2026:
1789:
1042:Lecha M, Puy H, Deybach JC (September 2009).
908:
640:
994:Proceedings of the Royal Society of Medicine
1093:Bruguera M, Herrero C (December 2005). "".
1037:
1035:
2033:
2019:
1316:Paumgartner G, Beuers U (September 2002).
1244:
1127:
722:Bonkovsky HL, Rudnick SR (December 2020).
266:will offer protection from visible light.
63:
35:
1766:
1725:
1612:
1585:
1535:
1518:Hansson M, Hederstedt L (February 1994).
1494:
1382:
1333:
1153:
1069:
1059:
1015:
1005:
964:
615:
531:
521:
477:"Autosomal erythropoietic protoporphyria"
391:
1633:
1275:The American Journal of Gastroenterology
1032:
861:"Acquired erythropoietic protoporphyria"
361:
117:
122:Acute photosensitivity reaction in EPP.
95:, leading to abnormally high levels of
2245:
1667:Journal of Inherited Metabolic Disease
1586:Ahmed jan, Marisa (13 December 2023).
990:"Photobiological aspects of porphyria"
987:
751:Journal of Inherited Metabolic Disease
366:Some over-the-counter drugs may help:
289:
87:(or commonly called EPP) is a form of
2014:
1613:Ahmed jan, Naila (16 December 2023).
557:Clinical and Experimental Dermatology
278:
1640:Arceci R, Hann IM, Smith OP (2006).
1477:Gorchein A, Foster GR (March 1999).
1262:from the original on 9 October 2019.
1230:Union Register of medicinal products
13:
1537:10.1111/j.1432-1033.1994.tb18615.x
818:The British Journal of Dermatology
435:St John's Institute of Dermatology
185:
14:
2274:
1865:
1408:Liver Transplantation and Surgery
1258:(Press release). 8 October 2019.
1256:U.S. Food and Drug Administration
1048:Orphanet Journal of Rare Diseases
510:World Journal of Gastroenterology
1768:10.1111/j.1365-2796.2010.02236.x
1644:. Malden, Mass.: Blackwell Pub.
1524:European Journal of Biochemistry
1384:10.1097/00005176-199611000-00006
1287:10.1111/j.1572-0241.2001.05363.x
1128:Anstey AV, Hift RJ (July 2007).
666:10.1111/j.1346-8138.2007.00386.x
402:
144:
1826:
1792:Zeitschrift fĂĽr Kinderheilkunde
1783:
1742:
1701:
1658:
1615:"Erythropoietic Protoporphyria"
1606:
1588:"Erythropoietic Protoporphyria"
1579:
1552:
1511:
1470:
1434:
1399:
1358:
1309:
1266:
1218:
1194:
1170:
1121:
1095:Gastroenterologia y Hepatologia
1086:
1044:"Erythropoietic protoporphyria"
981:
902:
852:
809:
411:
113:
2119:Hepatoerythropoietic porphyria
1561:Cellular and Molecular Biology
785:
742:
730:. Merck Sharp & Dohme Corp
728:Merck Manuals Consumer Version
715:
688:
591:
548:
469:
101:red blood cells (erythrocytes)
1:
2168:Hereditary hyperbilirubinemia
2154:Erythropoietic protoporphyria
1873:Erythropoietic protoporphyria
1847:10.1016/S0140-6736(61)92427-8
1107:10.1016/s0210-5705(05)71529-6
797:American Porphyria Foundation
462:
191:hepatocytes is secreted into
85:Erythropoietic protoporphyria
22:Erythropoietic protoporphyria
2090:Acute intermittent porphyria
1755:Journal of Internal Medicine
1456:10.1016/0016-5085(88)90627-0
966:10.1182/blood-2007-04-088120
457:List of cutaneous conditions
239:
210:
157:
7:
1621:. StatPearls Publishing LLC
988:Magnus IA (February 1968).
440:
344:Bone marrow transplantation
165:
44:Chronic skin lesions of EPP
10:
2279:
1226:"Scenesse (afamelanotide)"
1206:The Skin Cancer Foundation
1007:10.1177/003591576806100233
923:10.1016/j.jhep.2007.09.013
654:The Journal of Dermatology
617:10.1038/s41436-021-01176-z
424:
328:is an orally administered
2211:
2181:
2166:
2139:Hereditary coproporphyria
2127:
2098:
2073:
2049:
1973:
1887:
1679:10.1007/s10545-012-9544-4
1202:"Sun Protective Clothing"
880:10.1007/s00277-009-0859-7
763:10.1007/s10545-008-1037-0
354:, extracorporeal albumin
72:
48:
43:
34:
26:
21:
523:10.3748/wjg.v16.i36.4526
2203:Lucey–Driscoll syndrome
2198:Crigler–Najjar syndrome
2115:Porphyria cutanea tarda
1880:Genetics Home Reference
1444:Gas Naila troenterology
1335:10.1053/jhep.2002.36088
1146:10.1136/gut.2006.097576
452:Porphyria cutanea tarda
294:Several drugs are used
263:Sun protective clothing
2223:Dubin–Johnson syndrome
1061:10.1186/1750-1172-4-19
392:Experimental therapies
298:by patients with EPP:
123:
1496:10.1002/hep.510290314
1232:. European Commission
911:Journal of Hepatology
447:Xeroderma pigmentosum
362:Over-the-counter drug
348:liver transplantation
121:
2076:early mitochondrial:
2044:metabolism disorders
1642:Pediatric Hematology
1619:www.ncbi.nlm.nih.gov
1420:10.1002/lt.500050503
868:Annals of Hematology
604:Genetics in Medicine
303:Ursodeoxycholic acid
103:, plasma, skin, and
2149:Variegate porphyria
2130:late mitochondrial:
290:Off-label therapies
78:pain and irritation
2193:Gilbert's syndrome
1974:External resources
1804:10.1007/BF00438257
757:(Suppl 3): S1–S4.
279:Approved therapies
124:
2240:
2239:
2236:
2235:
2162:
2161:
2008:
2007:
1841:(7200): 448–451.
1651:978-1-4051-3400-2
1592:porphyrianews.com
1281:(12): 3468–3469.
1182:smartskincare.com
959:(12): 4108–4110.
830:10.1111/bjd.12242
697:Photo-Dermatology
569:10.1111/ced.12202
516:(36): 4526–4531.
247:Color temperature
176:protoporphyrin IX
82:
81:
16:Medical condition
2270:
2216:
2186:
2179:
2178:
2144:Harderoporphyria
2132:
2103:
2078:
2071:
2070:
2035:
2028:
2021:
2012:
2011:
1885:
1884:
1859:
1858:
1830:
1824:
1823:
1787:
1781:
1780:
1770:
1746:
1740:
1739:
1729:
1705:
1699:
1698:
1662:
1656:
1655:
1637:
1631:
1630:
1628:
1626:
1610:
1604:
1603:
1601:
1599:
1583:
1577:
1576:
1556:
1550:
1549:
1539:
1515:
1509:
1508:
1498:
1474:
1468:
1467:
1438:
1432:
1431:
1403:
1397:
1396:
1386:
1362:
1356:
1355:
1337:
1313:
1307:
1306:
1270:
1264:
1263:
1248:
1242:
1241:
1239:
1237:
1222:
1216:
1215:
1213:
1212:
1198:
1192:
1191:
1189:
1188:
1174:
1168:
1167:
1157:
1140:(7): 1009–1018.
1125:
1119:
1118:
1090:
1084:
1083:
1073:
1063:
1039:
1030:
1029:
1019:
1009:
985:
979:
978:
968:
944:
935:
934:
906:
900:
899:
865:
856:
850:
849:
824:(6): 1311–1315.
813:
807:
806:
804:
803:
789:
783:
782:
746:
740:
739:
737:
735:
719:
713:
712:
692:
686:
685:
649:
638:
637:
619:
610:(9): 1616–1623.
595:
589:
588:
552:
546:
545:
535:
525:
501:
492:
491:
489:
487:
473:
337:Activated carbon
68:
67:
39:
19:
18:
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2099:
2094:
2074:
2062:
2054:
2045:
2039:
2009:
2004:
2003:
1969:
1968:
1896:
1868:
1863:
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1831:
1827:
1788:
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1200:
1199:
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1184:
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1126:
1122:
1101:(10): 632–636.
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1040:
1033:
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907:
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2112:
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2081:
2079:
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2060:erythropoietic
2047:
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2038:
2037:
2030:
2023:
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2006:
2005:
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1990:
1978:
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1888:Classification
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1866:External links
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1489:(3): 995–996.
1469:
1450:(1): 177–181.
1433:
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1398:
1377:(4): 402–407.
1357:
1328:(3): 525–531.
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1000:(2): 196–198.
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341:
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326:Cholestyramine
323:
320:Plasmapheresis
317:
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219:Screening for
216:erythrocytes.
212:
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187:
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172:ferrochelatase
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97:protoporphyrin
93:ferrochelatase
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178:ring to form
177:
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145:Liver failure
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90:
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2100:
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1992:
1981:
1957:
1946:
1935:
1920:
1900:
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1838:
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1798:(1): 82–92.
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114:Presentation
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74:Risk factors
2214:conjugated:
437:in London.
378:B. subtilis
333:approaches.
310:mechanisms.
234:Wood’s lamp
59:dermatology
27:Other names
2253:Porphyrias
2247:Categories
1959:DiseasesDB
1483:Hepatology
1322:Hepatology
1236:8 December
1211:2022-01-26
1187:2019-04-02
802:2022-01-26
734:11 January
463:References
418:prevalence
397:Bitopertin
259:zinc oxide
255:sunscreens
139:gallstones
2173:bilirubin
2065:porphyrin
2051:Porphyria
1983:eMedicine
634:233719987
371:Proferrin
307:bile acid
296:off label
240:Treatment
229:cirrhotic
211:Diagnosis
199:400
158:Pregnancy
89:porphyria
50:Specialty
1994:Orphanet
1988:derm/473
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1855:13765301
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1812:13103364
1777:20412370
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1505:10189233
1428:10477837
1352:28282761
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1303:27973564
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1260:Archived
1164:17360790
1115:16373015
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626:33941881
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486:17 April
481:Orphanet
441:See also
356:dialysis
166:Genetics
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433:at the
425:History
314:Hematin
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953:Blood
892:S2CID
864:(PDF)
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678:S2CID
630:S2CID
581:S2CID
330:resin
305:is a
105:liver
2058:and
2042:Heme
1964:4484
1948:MeSH
1937:OMIM
1926:9-CM
1915:ILDS
1851:PMID
1808:PMID
1773:PMID
1732:PMID
1683:PMID
1646:ISBN
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221:FECH
193:bile
180:heme
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1800:doi
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