Abstract:Harlequin Ichthyosis is the most severe form of congenital Ichthyosis presenting at birth. It is characterized by thick, fissured armor-plate hyperkeratosis, ears and nose deformities, ectropion, eclabium with fish mouth appearance, flexion deformities of all joints and hypoplastic digits. It is a very rare disorder with autosomal recessive inheritance. Perinatal mortality is high and the survivors develop severe erythroderma subsequently. We report a case of Harlequin Ichthyosis not only because of its rarity… Show more
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