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Case report
Experience of low-dose dexamethasone use in the respiratory management of ichthyosis prematurity syndrome
  1. Davina Henderson1,
  2. Claire A Murphy1,2,
  3. Mary O'Dea1 and
  4. Michael A Boyle1,2
  1. 1Department of Neonatology, Rotunda Hospital Neonatal Unit, Dublin, Ireland
  2. 2Department of Paediatrics, Royal College of Surgeons in Ireland, Dublin, Ireland
  1. Correspondence to Dr Michael A Boyle; mboyle{at}rotunda.ie

Abstract

Ichthyosis prematurity syndrome (IPS) is a rare disorder of autosomal recessive inheritance. The cardinal features include prematurity, vernix like hyperkeratosis, eosinophilia and neonatal asphyxiation. This case report discusses the presentation and management of IPS. We aim to characterise the common features, the spectrum of disease within a single family and discuss a potential role for low-dose dexamethasone in the management of ventilator-dependent patients with IPS.

  • dermatology
  • congenital disorders
  • neonatal and paediatric intensive care

https://doi.org/10.1136/bcr-2021-243348

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    Footnotes

    • Contributors DH, CAM and MO'D evaluated the case, reviewed the literature, prepared the manuscript draft and contributed to the editing of the final draft. MAB identified the case and novel element, edited the initial manuscript, contributed to writing the manuscript and had final approval of the manuscript for submission. All authors have read and approved the manuscript.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Provenance and peer review Not commissioned; externally peer reviewed.