Efficacy of the melanocortin analogue Nle4-D-Phe7-α-melanocyte-stimulating hormone in the treatment of patients with Hailey-Hailey disease.
Bottom Line: Our results show that Nrf2 is an important target of the afamelanotide signalling that reduces oxidative stress.Because afamelanotide possesses antioxidant effects, we also assessed the clinical potential of this α-MSH analogue in the treatment of patients with HHD.Afamelanotide is effective for the treatment of skin lesions in HHD.
Affiliation: Porphyria Center, San Gallicano Institute IRCCS, Rome, Italy.Show MeSH
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Mentions: Sequencing of the entire ATP2C1 coding region and exon–intron boundaries identified a heterozygous mutation (c.457C > T in patient 4 and c.2395C > T in patient 5), resulting in a premature termination codon in both cases. Patient 4 had erythematous and crusted lesions located on her subclavicular, periscapular and substernal regions (Fig.2), while patient had scaly erythematous plaques on her umbilical area, right flank and inguinal fold. Previous topical treatment had given poor results.
Affiliation: Porphyria Center, San Gallicano Institute IRCCS, Rome, Italy.