Two for two: Dual therapy with erlotinib and acitretin for twins with severe keratoderma in Olmsted syndrome

Sneha Butala; Sheshanna Phan; Dawn H. Siegel; Valerie Carlberg; Amy S. Paller

doi:10.1111/pde.15264

Two for two: Dual therapy with erlotinib and acitretin for twins with severe keratoderma in Olmsted syndrome

Sneha Butala, Sheshanna Phan, Dawn H. Siegel, Valerie Carlberg, Amy S. Paller^*

^*Corresponding author for this work

Dermatology

Research output: Contribution to journal › Article › peer-review

Abstract

Olmsted syndrome (OS) is a rare genetic disorder, characterized by painful palmoplantar keratoderma (PPK), periorificial and intertriginous hyperkeratoses, and alopecia. Fewer than 75 cases have been described. Variants in TRPV3 result in constitutive activation of transient receptor potential vanilloid 3, leading to increased epidermal growth factor receptor (EGFR) signaling, palmoplantar epidermal hyperproliferation, and exquisite lesional pain. We describe pre-school aged twins with OS with partial improvement from oral erlotinib, an EGFR inhibitor, but dramatic reduction of their persistent palmoplantar thickening and pain from adding acitretin.

Original language	English (US)
Journal	Pediatric dermatology
DOIs	https://doi.org/10.1111/pde.15264
State	Accepted/In press - 2023

Keywords

Olmsted syndrome
acitretin
erlotinib
keratoderma
pediatric dermatology

ASJC Scopus subject areas

Pediatrics, Perinatology, and Child Health
Dermatology

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10.1111/pde.15264

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title = "Two for two: Dual therapy with erlotinib and acitretin for twins with severe keratoderma in Olmsted syndrome",

abstract = "Olmsted syndrome (OS) is a rare genetic disorder, characterized by painful palmoplantar keratoderma (PPK), periorificial and intertriginous hyperkeratoses, and alopecia. Fewer than 75 cases have been described. Variants in TRPV3 result in constitutive activation of transient receptor potential vanilloid 3, leading to increased epidermal growth factor receptor (EGFR) signaling, palmoplantar epidermal hyperproliferation, and exquisite lesional pain. We describe pre-school aged twins with OS with partial improvement from oral erlotinib, an EGFR inhibitor, but dramatic reduction of their persistent palmoplantar thickening and pain from adding acitretin.",

keywords = "Olmsted syndrome, acitretin, erlotinib, keratoderma, pediatric dermatology",

author = "Sneha Butala and Sheshanna Phan and Siegel, {Dawn H.} and Valerie Carlberg and Paller, {Amy S.}",

note = "Publisher Copyright: {\textcopyright} 2023 The Authors. Pediatric Dermatology published by Wiley Periodicals LLC.",

year = "2023",

doi = "10.1111/pde.15264",

language = "English (US)",

journal = "Pediatric Dermatology",

issn = "0736-8046",

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T2 - Dual therapy with erlotinib and acitretin for twins with severe keratoderma in Olmsted syndrome

AU - Butala, Sneha

AU - Phan, Sheshanna

AU - Siegel, Dawn H.

AU - Carlberg, Valerie

AU - Paller, Amy S.

PY - 2023

Y1 - 2023

N2 - Olmsted syndrome (OS) is a rare genetic disorder, characterized by painful palmoplantar keratoderma (PPK), periorificial and intertriginous hyperkeratoses, and alopecia. Fewer than 75 cases have been described. Variants in TRPV3 result in constitutive activation of transient receptor potential vanilloid 3, leading to increased epidermal growth factor receptor (EGFR) signaling, palmoplantar epidermal hyperproliferation, and exquisite lesional pain. We describe pre-school aged twins with OS with partial improvement from oral erlotinib, an EGFR inhibitor, but dramatic reduction of their persistent palmoplantar thickening and pain from adding acitretin.

AB - Olmsted syndrome (OS) is a rare genetic disorder, characterized by painful palmoplantar keratoderma (PPK), periorificial and intertriginous hyperkeratoses, and alopecia. Fewer than 75 cases have been described. Variants in TRPV3 result in constitutive activation of transient receptor potential vanilloid 3, leading to increased epidermal growth factor receptor (EGFR) signaling, palmoplantar epidermal hyperproliferation, and exquisite lesional pain. We describe pre-school aged twins with OS with partial improvement from oral erlotinib, an EGFR inhibitor, but dramatic reduction of their persistent palmoplantar thickening and pain from adding acitretin.

KW - Olmsted syndrome

KW - acitretin

KW - erlotinib

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KW - pediatric dermatology

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Two for two: Dual therapy with erlotinib and acitretin for twins with severe keratoderma in Olmsted syndrome

Abstract

Keywords

ASJC Scopus subject areas

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