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Dupilumab Improves Pruritus in Netherton Syndrome: A Case Study
The patient was a 26-year-old male. He had red and scaling skin of the entire body since birth, as well as an elevated level of serum IgE. Genetic testing revealed a mutation in the SPINK5 gene, which had confirmed the diagnosis with Netherton syndrome. He has had significant pruritis since birth, a...
Autores principales: | , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8946880/ https://www.ncbi.nlm.nih.gov/pubmed/35327681 http://dx.doi.org/10.3390/children9030310 |
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author | Inaba, Yutaka Kanazawa, Nobuo Muraoka, Kyoko Yariyama, Azusa Kawaguchi, Ami Kunimoto, Kayo Kaminaka, Chikako Yamamoto, Yuki Tsujioka, Kaoru Yoshida, Akira Yanagi, Teruki Jinnin, Masatoshi |
author_facet | Inaba, Yutaka Kanazawa, Nobuo Muraoka, Kyoko Yariyama, Azusa Kawaguchi, Ami Kunimoto, Kayo Kaminaka, Chikako Yamamoto, Yuki Tsujioka, Kaoru Yoshida, Akira Yanagi, Teruki Jinnin, Masatoshi |
author_sort | Inaba, Yutaka |
collection | PubMed |
description | The patient was a 26-year-old male. He had red and scaling skin of the entire body since birth, as well as an elevated level of serum IgE. Genetic testing revealed a mutation in the SPINK5 gene, which had confirmed the diagnosis with Netherton syndrome. He has had significant pruritis since birth, and subsequently had symptoms of sleeping disorders and concentration difficulty throughout the day. Since treatment with various antihistamines were not effective, we administered dupilumab and found that it was effective in immediate elimination of pruritus and gradual reduction of the rash. Dupilumab has been administered for one year without any adverse events or recurrence of symptoms. Although studies have previously described cases who used dupilumab for Netherton syndrome, reported effects have been limited or transient. Additional studies are needed to confirm the effect of dupilumab for Netherton syndrome, which currently lack any effective treatment strategies. |
format | Online Article Text |
id | pubmed-8946880 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-89468802022-03-25 Dupilumab Improves Pruritus in Netherton Syndrome: A Case Study Inaba, Yutaka Kanazawa, Nobuo Muraoka, Kyoko Yariyama, Azusa Kawaguchi, Ami Kunimoto, Kayo Kaminaka, Chikako Yamamoto, Yuki Tsujioka, Kaoru Yoshida, Akira Yanagi, Teruki Jinnin, Masatoshi Children (Basel) Case Report The patient was a 26-year-old male. He had red and scaling skin of the entire body since birth, as well as an elevated level of serum IgE. Genetic testing revealed a mutation in the SPINK5 gene, which had confirmed the diagnosis with Netherton syndrome. He has had significant pruritis since birth, and subsequently had symptoms of sleeping disorders and concentration difficulty throughout the day. Since treatment with various antihistamines were not effective, we administered dupilumab and found that it was effective in immediate elimination of pruritus and gradual reduction of the rash. Dupilumab has been administered for one year without any adverse events or recurrence of symptoms. Although studies have previously described cases who used dupilumab for Netherton syndrome, reported effects have been limited or transient. Additional studies are needed to confirm the effect of dupilumab for Netherton syndrome, which currently lack any effective treatment strategies. MDPI 2022-02-24 /pmc/articles/PMC8946880/ /pubmed/35327681 http://dx.doi.org/10.3390/children9030310 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Case Report Inaba, Yutaka Kanazawa, Nobuo Muraoka, Kyoko Yariyama, Azusa Kawaguchi, Ami Kunimoto, Kayo Kaminaka, Chikako Yamamoto, Yuki Tsujioka, Kaoru Yoshida, Akira Yanagi, Teruki Jinnin, Masatoshi Dupilumab Improves Pruritus in Netherton Syndrome: A Case Study |
title | Dupilumab Improves Pruritus in Netherton Syndrome: A Case Study |
title_full | Dupilumab Improves Pruritus in Netherton Syndrome: A Case Study |
title_fullStr | Dupilumab Improves Pruritus in Netherton Syndrome: A Case Study |
title_full_unstemmed | Dupilumab Improves Pruritus in Netherton Syndrome: A Case Study |
title_short | Dupilumab Improves Pruritus in Netherton Syndrome: A Case Study |
title_sort | dupilumab improves pruritus in netherton syndrome: a case study |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8946880/ https://www.ncbi.nlm.nih.gov/pubmed/35327681 http://dx.doi.org/10.3390/children9030310 |
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