Hair Improvement in <scp>SPINK5</scp> ‐ <scp>sEDD</scp> (Netherton Syndrome) With Dupilumab

SPINK5-syndromic epidermal differentiation disorder (sEDD) (formerly known as Netherton syndrome) is a rare genodermatosis characterized by ichthyosis linearis circumflexa, atopic diathesis, and trichorrhexis invaginata. We report an 11-year-old girl with SPINK5-sEDD who experienced marked improvement in hair density, texture, and pruritus following dupilumab. The early and sustained hair response suggests that IL-4/IL-13 blockade may modulate the perifollicular inflammatory environment and enhance follicular function. This observation supports emerging evidence that dupilumab may confer hair benefits in SPINK5-sEDD and underscores the need for systematic evaluation of this effect.

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Publication Details

Journal
Pediatric Dermatology
Published
2026-04-13
DOI
https://doi.org/10.1111/pde.70194
Primary Topic
Skin and Cellular Biology Research
Type
article
Field-Weighted Citation Impact
0.00
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article

Hair Improvement in SPINK5 ‐ sEDD (Netherton Syndrome) With Dupilumab

A. Sousa Pedrosa, Alberto Mota, Bárbara Vieira Granja, Manuel Bernardo Costa et al.
Pediatric Dermatology
Skin and Cellular Biology Research
article

Hair Improvement in SPINK5 ‐ sEDD (Netherton Syndrome) With Dupilumab

A. Sousa Pedrosa, Alberto Mota, Bárbara Vieira Granja, Manuel Bernardo Costa, Rafael Baptista Santos
article en

Abstract

SPINK5-syndromic epidermal differentiation disorder (sEDD) (formerly known as Netherton syndrome) is a rare genodermatosis characterized by ichthyosis linearis circumflexa, atopic diathesis, and trichorrhexis invaginata. We report an 11-year-old girl with SPINK5-sEDD who experienced marked improvement in hair density, texture, and pruritus following dupilumab. The early and sustained hair response suggests that IL-4/IL-13 blockade may modulate the perifollicular inflammatory environment and enhance follicular function. This observation supports emerging evidence that dupilumab may confer hair benefits in SPINK5-sEDD and underscores the need for systematic evaluation of this effect.

Pediatric Dermatology
Universidade do Porto (PT), Hospital de São João (PT), Administração Regional de Saúde de Lisboa e Vale do Tejo (PT)
Openalex Percentile: Top 14%
Skin and Cellular Biology Research
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