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

4 Scopus citations

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)
Pages (from-to)	735-737
Number of pages	3
Journal	Pediatric dermatology
Volume	40
Issue number	4
DOIs	https://doi.org/10.1111/pde.15264
State	Published - Jul 1 2023

Keywords

Olmsted syndrome
acitretin
erlotinib
keratoderma
pediatric dermatology

ASJC Scopus subject areas

Dermatology
Pediatrics, Perinatology, and Child Health

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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",

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AU - Butala, Sneha

AU - Phan, Sheshanna

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AU - Carlberg, Valerie

AU - Paller, Amy S.

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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.

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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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