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.


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*


  • Dexamethasone

Supplementary concepts

  • Ichthyosis prematurity syndrome