Abstract

Introduction: Harlequin ichthyosis is one of the most devastating of the genodermatoses. Neonates usually die within the first few days of life from infection or dehydration related complications. Prenatal diagnosis remains difficult but may be possible in high risk pregnancies by performing a fetal skin biopsy or by three-dimensional ultrasonography. Case Report: We report the case of Harlequin ichthyosis for its rarity and briefly review literature. Conclusion: This case has been reported for the rarity of Harlequin ichthyosis and to create awareness among pediatricians to identify the condition promptly.

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