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...

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Bibliographic Details
Published in:Children (Basel) 2022-02, Vol.9 (3), p.310
Main Authors: Inaba, Yutaka, Kanazawa, Nobuo, Muraoka, Kyoko, Yariyama, Azusa, Kawaguchi, Ami, Kunimoto, Kayo, Kaminaka, Chikako, Yamamoto, Yuki, Tsujioka, Kaoru, Yoshida, Akira, Yanagi, Teruki, Jinnin, Masatoshi
Format: Article
Language:English
Subjects:
atopic dermatitis
Case Report
Chemokines
Congenital diseases
Dermatitis
dupilumab
Eczema
Erythema
Females
Histamine
Histopathology
Hyperplasia
Monoclonal antibodies
Netherton syndrome
Patients
Pediatrics
Pruritus
Skin
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Summary: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.
ISSN:2227-9067
2227-9067
DOI:10.3390/children9030310