CASE REPORT article
Front. Immunol.
Sec. Autoimmune and Autoinflammatory Disorders : Autoimmune Disorders
Volume 16 - 2025 | doi: 10.3389/fimmu.2025.1647441
This article is part of the Research TopicNew Insights into Inflammation Driven Autoimmune Skin Disorders: Trends and ChallengesView all 10 articles
Dupilumab Treatment for Chinese Nagashima-type Palmoplantar Keratoderma Associated with Atopic Dermatitis: a Case Report
Provisionally accepted
- 1Department of Dermatology, Sir Run Run Shaw Hospital, Hangzhou, China
- 2Zhejiang University School of Medicine Sir Run Run Shaw Hospital, Hangzhou, China
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Patients with Nagashima-type palmoplantar keratoderma (NPPK) experience progressive, painful hyperkeratosis and fissuring of palms and soles that limits daily activities Due to the incomplete understanding of its pathogenesis, there are currently no effective treatments for NPPK. We report a 26-year-old woman with lifelong, worsening palmoplantar keratoderma, nail dystrophy, and concomitant atopic dermatitis refractory to topical treatments. Next-generation sequencing revealed compound heterozygous mutations in SERPINB7 (c.796C>T, p.Arg266Ter) and filaggrin (FLG, c.3321delA, p.Gly1109GlufsTer13), while her asymptomatic parents and brother carried only single heterozygous variants, underscoring the digenic pathogenesis in our patient. After 42 weeks of dupilumab treatment, palmoplantar keratosis and nail changes had almost completely resolved, and the eruption resembled mild chronic eczema. Dupilumab therefore appears to be a safe and effective option for digenic NPPK complicated by atopic dermatitis and warrants further investigation in larger cohorts.
Keywords: Chinese Nagashima-type Palmoplantar Keratoderma, atopic dermatitis, Dupilumab, SERPINB7, FLG
Received: 15 Jun 2025; Accepted: 09 Sep 2025.
Copyright: © 2025 Hua, Cheng and Chen. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Xianzhen Chen, Zhejiang University School of Medicine Sir Run Run Shaw Hospital, Hangzhou, China
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