Experience of low-dose dexamethasone use in the respiratory management of ichthyosis prematurity syndrome

Davina Henderson; Claire A Murphy; Mary O'Dea; Michael A Boyle

doi:10.1136/bcr-2021-243348

Experience of low-dose dexamethasone use in the respiratory management of ichthyosis prematurity syndrome

BMJ Case Rep. 2021 Aug 20;14(8):e243348. doi: 10.1136/bcr-2021-243348.

Authors

Davina Henderson¹, Claire A Murphy^{1

2}, Mary O'Dea¹, Michael A Boyle^{3

2}

Affiliations

¹ Department of Neonatology, Rotunda Hospital Neonatal Unit, Dublin, Ireland.
² Department of Paediatrics, Royal College of Surgeons in Ireland, Dublin, Ireland.
³ Department of Neonatology, Rotunda Hospital Neonatal Unit, Dublin, Ireland mboyle@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.

Keywords: congenital disorders; dermatology; neonatal and paediatric intensive care.

Publication types

Case Reports

MeSH terms

Dexamethasone / therapeutic use
Humans
Ichthyosis*
Infant, Newborn
Infant, Premature, Diseases* / drug therapy
Keratosis*

Substances

Dexamethasone

Supplementary concepts

Ichthyosis prematurity syndrome