Harlequin Ichthyosis: Folklore of Demon

Background: Harlequin ichthyosis (HI) is a severe and rare autosomal recessive congenital ichthyosis, characterized by hyperkeratosis, extensive fissuring massive, diamond-shaped scales which limit the child’s movements. The term harlequin comes from baby’s facial expression pattern of like 17th century entertainers, harlequins. underlying genetic abnormality has been identified as mutation in lipid-transporter gene ABCA 12 on chromosome 2q35. Aim objectives: To provide comprehensive knowledge forms abolish superstitious notion regarding appearance neonate. Materials Methods: present report case premature girl baby delivered at MIMS, Mandya. neonate presented plaques rigid fixed skin, separated deep red fissures, features were obliterated thickened undeveloped nose pinna, ectropion eclabium. Intensive care was given survival prolonged systemic retinoids. succumbed to respiratory failure few days later. Results conclusion: study gives provides emphasis sensitization disease prevent any misconception or superstition. Also lays Genetic counseling couple further occurrences. KEY WORDS: Eclabium, Ectropion, ichthyosis.