Management focuses on fluids and emollients
Management of congenital ichthyosis involves adequate pulmonary support, as the scaling and/or plate formation may cause a restrictive ventilatory defect; the use of humidified incubators; fluid and electrolyte replacement; emollients and wet compresses to maintain skin moisture and prevent further scaling or tautness of the membrane; ophthalmologic consultation to manage ectropion; and the treatment of infection.4,5 Oral retinoids are used in select cases.14
The prognosis varies with disease severity, but complications include infection, temperature instability, and dehydration secondary to skin barrier dysfunction. Most collodion babies survive to adulthood while few, if any, babies affected with harlequin ichthyosis survive the neonatal period. Ongoing research in corrective gene transfer provides hope for future therapy.15
Our patient required a humidified incubator
Following a consult with our hospital neonatologist, we closely monitored our patient’s volume status and electrolytes, placed him in a humidified incubator, and ensured that he was treated with emollient creams. Our patient was also treated for neonatal jaundice.
We obtained a consultation with a genetics counselor, who agreed with our diagnosis and ordered appropriate genetic testing. The patient has since been lost to follow-up.
Correspondence
T. Aaron Zeller, MD, 155 Academy Avenue, Greenwood, SC 29646; azeller@selfregional.org