A Melanocortin-4 Receptor Agonist Induces Skin and Hair Pigmentation in Patients with Monogenic Mutations in the Leptin-Melanocortin Pathway.

Hyperpigmentation Leptin receptor Leptin-melanocortin pathway Melanocortin-1 receptor Melanocortin-4 receptor agonist Proopiomelanocortin

Journal

Skin pharmacology and physiology
ISSN: 1660-5535
Titre abrégé: Skin Pharmacol Physiol
Pays: Switzerland
ID NLM: 101188418

Informations de publication

Date de publication:
2021
Historique:
received: 21 10 2020
accepted: 25 03 2021
pubmed: 1 6 2021
medline: 14 1 2022
entrez: 31 5 2021
Statut: ppublish

Résumé

Gene mutations within the leptin-melanocortin signaling pathway lead to severe early-onset obesity. Recently, a phase 2 trial evaluated new pharmacological treatment options with the MC4R agonist setmelanotide in patients with mutations in the genes encoding proopiomelanocortin (POMC) and leptin receptor (LEPR). During treatment with setmelanotide, changes in skin pigmentation were observed, probably due to off-target effects on the closely related melanocortin 1 receptor (MC1R). Here, we describe in detail the findings of dermatological examinations and measurements of skin pigmentation during this treatment over time and discuss the impact of these changes on patient safety. In an investigator-initiated, phase 2, open-label pilot study, 2 patients with loss-of-function POMC gene mutations and 3 patients with loss-of-function variants in LEPR were treated with the MC4R agonist setmelanotide. Dermatological examination, dermoscopy, whole body photographic documentation, and spectrophotometric measurements were performed at screening visit and approximately every 3 months during the course of the study. We report the results of a maximum treatment duration of 46 months. Skin pigmentation increased in all treated patients, as confirmed by spectrophotometry. During continuous treatment, the current results indicate that elevated tanning intensity levels may stabilize over time. Lips and nevi also darkened. In red-haired study participants, hair color changed to brown after initiation of setmelanotide treatment. Setmelanotide treatment leads to skin tanning and occasionally hair color darkening in both POMC- and LEPR-deficient patients. No malignant skin changes were observed in the patients of this study. However, the results highlight the importance of regular skin examinations before and during MC4R agonist treatment.

Sections du résumé

BACKGROUND AND OBJECTIVES OBJECTIVE
Gene mutations within the leptin-melanocortin signaling pathway lead to severe early-onset obesity. Recently, a phase 2 trial evaluated new pharmacological treatment options with the MC4R agonist setmelanotide in patients with mutations in the genes encoding proopiomelanocortin (POMC) and leptin receptor (LEPR). During treatment with setmelanotide, changes in skin pigmentation were observed, probably due to off-target effects on the closely related melanocortin 1 receptor (MC1R). Here, we describe in detail the findings of dermatological examinations and measurements of skin pigmentation during this treatment over time and discuss the impact of these changes on patient safety.
METHODS METHODS
In an investigator-initiated, phase 2, open-label pilot study, 2 patients with loss-of-function POMC gene mutations and 3 patients with loss-of-function variants in LEPR were treated with the MC4R agonist setmelanotide. Dermatological examination, dermoscopy, whole body photographic documentation, and spectrophotometric measurements were performed at screening visit and approximately every 3 months during the course of the study.
RESULTS RESULTS
We report the results of a maximum treatment duration of 46 months. Skin pigmentation increased in all treated patients, as confirmed by spectrophotometry. During continuous treatment, the current results indicate that elevated tanning intensity levels may stabilize over time. Lips and nevi also darkened. In red-haired study participants, hair color changed to brown after initiation of setmelanotide treatment.
DISCUSSION CONCLUSIONS
Setmelanotide treatment leads to skin tanning and occasionally hair color darkening in both POMC- and LEPR-deficient patients. No malignant skin changes were observed in the patients of this study. However, the results highlight the importance of regular skin examinations before and during MC4R agonist treatment.

Identifiants

pubmed: 34058738
pii: 000516282
doi: 10.1159/000516282
doi:

Substances chimiques

Leptin 0
Melanocortins 0
Receptor, Melanocortin, Type 4 0

Types de publication

Clinical Trial, Phase II Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

307-316

Informations de copyright

© 2021 S. Karger AG, Basel.

Auteurs

Varvara Kanti (V)

Berlin Institute of Health, Department of Dermatology and Allergy, Charité, Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Clinical Research Center for Hair and Skin Science, Berlin, Germany.

Lia Puder (L)

Institute for Experimental Pediatric Endocrinology, Berlin Institute of Health, Charité, Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin, Germany.
Berlin Institute of Health, Department for Pediatric Endocrinology and Diabetology, Charité, Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin, Germany.

Irina Jahnke (I)

Berlin Institute of Health, Department of Dermatology and Allergy, Charité, Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Clinical Research Center for Hair and Skin Science, Berlin, Germany.

Philipp Maximilian Krabusch (PM)

Institute for Experimental Pediatric Endocrinology, Berlin Institute of Health, Charité, Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin, Germany.
Berlin Institute of Health, Department for Pediatric Endocrinology and Diabetology, Charité, Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin, Germany.

Jan Kottner (J)

Berlin Institute of Health, Department of Dermatology and Allergy, Charité, Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Clinical Research Center for Hair and Skin Science, Berlin, Germany.

Annika Vogt (A)

Berlin Institute of Health, Department of Dermatology and Allergy, Charité, Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Clinical Research Center for Hair and Skin Science, Berlin, Germany.

Claudia Richter (C)

Berlin Institute of Health, Department of Dermatology and Allergy, Charité, Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Clinical Research Center for Hair and Skin Science, Berlin, Germany.

Annette Andruck (A)

Berlin Institute of Health, Department of Dermatology and Allergy, Charité, Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Clinical Research Center for Hair and Skin Science, Berlin, Germany.

Lara Lechner (L)

Institute for Experimental Pediatric Endocrinology, Berlin Institute of Health, Charité, Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin, Germany.

Christine Poitou (C)

Assistance Publique Hôpitaux de Paris, Pitié-Salpêtrière Hospital, CRNH-Ile de France Paris, Nutrition department, Paris, France.
Sorbonne Université, INSERM, Nutrition and Obesity, systemic approach (NutriOmics) research group, Paris, France.

Heiko Krude (H)

Institute for Experimental Pediatric Endocrinology, Berlin Institute of Health, Charité, Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin, Germany.

Keith Gottesdiener (K)

Rhythm Pharmaceuticals, Boston, Massachusetts, USA.

Karine Clément (K)

Assistance Publique Hôpitaux de Paris, Pitié-Salpêtrière Hospital, CRNH-Ile de France Paris, Nutrition department, Paris, France.
Sorbonne Université, INSERM, Nutrition and Obesity, systemic approach (NutriOmics) research group, Paris, France.

Ismaa Sadaf Farooqi (IS)

Wellcome Trust-MRC Institute of Metabolic Science, University of Cambridge Metabolic Research Laboratories and NIHR Cambridge Biomedical Research Centre, Addenbrooke's Hospital, Cambridge, United Kingdom.

Susanna Wiegand (S)

Berlin Institute of Health, Center for Social-Pediatric Care/Pediatric Endocrinology and Diabetology, Charité, Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin, Germany.

Peter Kühnen (P)

Institute for Experimental Pediatric Endocrinology, Berlin Institute of Health, Charité, Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin, Germany.

Ulrike Blume-Peytavi (U)

Berlin Institute of Health, Department of Dermatology and Allergy, Charité, Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Clinical Research Center for Hair and Skin Science, Berlin, Germany.

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Classifications MeSH