Harlequin fetus

This code represents Harlequin ichthyosis, a severe and rare genetic skin disorder present at birth. Infants with this condition are born with thick, plate-like scales covering their entire body, often leading to severe ectropion, eclabium, and respiratory distress. It is the most severe form of congenital ichthyosis.

Assign this code for documented cases of Harlequin ichthyosis in newborns or infants. Clinical documentation should explicitly state "Harlequin fetus" or "Harlequin ichthyosis" as the diagnosis. This code is typically used in the perinatal period or early infancy.

• Ensure documentation clearly distinguishes Harlequin ichthyosis from other forms of congenital ichthyosis.
• Consider additional codes for associated complications such as respiratory failure (e.g., P28.5), sepsis (e.g., P36.-), or feeding difficulties (e.g., P92.-).
• This is a congenital anomaly code, so it will typically be a primary diagnosis in the neonatal period.