Medical Care

Airway Maintenance

In treating this severe and very rare genetic skin disorder (harlequin ichthyosis) upon delivery, the baby’s airway and circulation is the foremost priority. The healthcare team must ensure that the baby’s/patient's airway, breathing, and circulation are in stable condition after delivery. The baby will require intravenous access. It will be through the access where the medication will be given. Peripheral access may be difficult. Umbilical cannulation may be necessary. The infant must be placed in a humidified incubator, the temperature, respiratory rate, heart rate and oxygen saturation must be monitored by the healthcare team. Once the vital sign have stabilized the baby is then transferred into a level 3 neonatal nursery station or room.

Projection of Conjunctiva

Expose keratitis results from ectropian of the eye-side. It is necessary to apply ophthalmic lubricants frequently to protect the conjunctivae. It is advisable to bathe the infants twice daily and use frequent wet sodium chloride compresses followed by application of bland lubricants to soften hard skin. Topical keratolytics (eg, salicylic acid) are not recommended in newborns because of potential systemic toxicity.

Intravenous Access for Feeding

It is prescribed to have intravenous access for intravenous fluids as it is almost always required. Neonates with harlequin ichthyosis initially do not feed well. There is a need to calculate daily fluid requirements since the neonates will experience excess cutaneous water losses. In addition to that, monitoring of serum electrolyte levels is required. There also having a high risk of hypernatremic dehydration.

Sterile Environment

The sterile environment has to be always maintained to avoid infection. Frequent cultures of the skin should be taken. Growth of pathogenic organisms (eg, Staphylococcus aureus, Pseudomonas aeruginosa) indicates risk of sepsis. The blood culture has to be drawn as sepsis can occur quickly in affected infants. A consensus does not exist regarding the use of prophylactic antibiotics in these patients.