Influence of meteorological data on sun tolerance in patients with erythropoietic protoporphyria in France

Bataille, S. de; Dutartre, H.; Puy, Hervé; Deybach, Jean Charles; Gouya, Laurent; Raffray, E.; Pithon, M.; Stalder, J.‐F.; Nguyen, Jean–Michel; Barbarot, S.

doi:10.1111/bjd.14600

Cited by 12 publications

(8 citation statements)

References 35 publications

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“…We need to follow-up the PP level of incomplete EPP carefully in the future. A previous report found that 40% of EPP patients showed an improvement in their symptoms, following a build-up of sunlight tolerance after repeated exposure (42). EPP patients seem to benefit from UV 'desensitization' therapy, and anecdotal evidence suggests that natural sunlight exposure improves light tolerance (43).…”

Section: Clinical Features and Course In Japanese Erythropoietic Protmentioning

confidence: 98%

“…Afamelanotide, a potent α-melanocyte-stimulating hormone (α-MSH) analogue, was approved for treatment of EPP by the European Medicines Agency in 2014. Afamelanotide can darken the skin colour and increase tolerance to sunlight exposure without pain (42,43). Afamelanotideinduced eumelanin synthesis provided photoprotection and decreased the consequences of phototoxicity (46).…”

Section: Clinical Features and Course In Japanese Erythropoietic Protmentioning

confidence: 99%

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Erythropoietic Protoporphyria in a Japanese Population

Mizawa

Makino²,

Nakano³

et al. 2019

Acta Derm Venerol

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Section: Clinical Features and Course In Japanese Erythropoietic Protmentioning

confidence: 98%

Section: Clinical Features and Course In Japanese Erythropoietic Protmentioning

confidence: 99%

Erythropoietic Protoporphyria in a Japanese Population

Mizawa

Makino²,

Nakano³

et al. 2019

Acta Derm Venerol

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“…It is extremely challenging to investigate the efficacy of a potentially beneficial treatment in EPP because, as Corbett et al stated in 1977, it is difficult to assess the efficacy of treatment in a disease where signs and symptoms are provoked by sunlight. Light exposure is unpredictable in many climates [17] and symptoms can be prevented by avoiding light. Moreover, results of artificial irradiation tests are unreliable, because the intolerance of individual patients to light varies from one occasion to another, even in the absence of treatment, and some patients show consistently negative tests when artificial light is used [18].…”

Section: Overview Of Treatment Optionsmentioning

confidence: 99%

Afamelanotide for prevention of phototoxicity in erythropoietic protoporphyria

Wensink

Wagenmakers

Langendonk

2021

Expert Review of Clinical Pharmacology

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Introduction: In erythropoietic protoporphyria (EPP), an inherited disorder of heme biosynthesis, accumulation of protoporphyrin IX results in acute phototoxicity. EPP patients experience severe burning pain after light exposure, which results in a markedly reduced quality of life. Afamelanotide is the first effective approved medical treatment for EPP, acting on melanocortin-1 receptors. This article aims to review afamelanotide. Areas covered: This review summarizes the chemical properties, pharmacokinetics, safety, preclinical and clinical data on afamelanotide in EPP, and post-marketing surveillance. PubMed search, manufacturers' websites, and relevant articles used for approval by authorities were used for the literature search. Expert opinion: Afamelanotide is an α-melanocyte-stimulating hormone analog. It can activate eumelanogenesis without exposure to UV radiation. Clinical studies in EPP showed that afamelanotide treatment significantly increased exposure to sunlight and QoL. In our clinical experience afamelanotide treatment is much more effective in clinical practice than demonstrated in clinical trials and should be made available for all EPP patients meeting inclusion criteria. The 60-day interval period was not based on effectiveness studies, and therefore for some of the patients the maximum of four implants per year with the 60-day interval is insufficient. Afamelanotide is well tolerated; common adverse events were headache, fatigue, and nausea.

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“…Also, study is difficult, particularly because people with EPP become frightened of sunlight exposure, so in the randomised studies (Europe and the USA) patients may still have gone out less than they would have been able to for fear of painful reactions, so this study endpoint might not have fully reflected the benefits of afamelanotide. Conversely, perhaps many of those who received afamelanotide guessed they were on active treatment because of tanning and so went out longer (so possibly gaining benefit through greater exposure to sunlight 38 ).…”

Section: Acute Phototoxic Symptomsmentioning

confidence: 99%

An overview of the cutaneous porphyrias

Dawe

2017

F1000Res

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This is an overview of the cutaneous porphyrias. It is a narrative review based on the published literature and my personal experience; it is not based on a formal systematic search of the literature. The cutaneous porphyrias are a diverse group of conditions due to inherited or acquired enzyme defects in the porphyrin–haem biosynthetic pathway. All the cutaneous porphyrias can have (either as a consequence of the porphyria or as part of the cause of the porphyria) involvement of other organs as well as the skin. The single commonest cutaneous porphyria in most parts of the world is acquired porphyria cutanea tarda, which is usually due to chronic liver disease and liver iron overload. The next most common cutaneous porphyria, erythropoietic protoporphyria, is an inherited disorder in which the accumulation of bile-excreted protoporphyrin can cause gallstones and, rarely, liver disease. Some of the porphyrias that cause blistering (usually bullae) and fragility (clinically and histologically identical to porphyria cutanea tarda) can also be associated with acute neurovisceral porphyria attacks, particularly variegate porphyria and hereditary coproporphyria. Management of porphyria cutanea tarda mainly consists of visible-light photoprotection measures while awaiting the effects of treating the underlying liver disease (if possible) and treatments to reduce serum iron and porphyrin levels. In erythropoietic protoporphyria, the underlying cause can be resolved only with a bone marrow transplant (which is rarely justifiable in this condition), so management consists particularly of visible-light photoprotection and, in some countries, narrowband ultraviolet B phototherapy. Afamelanotide is a promising and newly available treatment for erythropoietic protoporphyria and has been approved in Europe since 2014.

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Influence of meteorological data on sun tolerance in patients with erythropoietic protoporphyria in France

Abstract: This study proposes a benefit of natural progressive sun exposure for patients with EPP.

Cited by 12 publications

References 35 publications

Erythropoietic Protoporphyria in a Japanese Population

Erythropoietic Protoporphyria in a Japanese Population

Afamelanotide for prevention of phototoxicity in erythropoietic protoporphyria

An overview of the cutaneous porphyrias

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