It is an autosomal recessive, and occasionally autosomal dominant mutant extremely rare disorder with only 100 reported case in literature. This fatal disorder occur in both sexes and all races. In most circumstances the newborn die soon after birth Also it is known as harlequin fetus, alligator baby or keratosis diffusa fatalis.

Harlequin ichthyosis is the most severe form of congenital ichthyoses with horny (Keratinized) and chapped thick skin throughout the body. In this disease, disruption in keratinization of epidermis results in the impaired barrier function and susceptibility of the neonate to dehydration and infection. In this article, a term male neonate with harlequin ichthyosis, born of a 29-year-old mother, was reported. On initial examination by a pediatrician, thick skin was observed with deep transverse clefts and hyper keratinization, and the infant was the infant was discharged because of absence of respiratory disorder and regularity of heart rate.

Introduction: Lamellar ichthyosis (collodion baby) is a cornification disorder classified under the category of autosomal recessive congenital ichthyosis and characterized by hyperkeratosis. Early-stage retinoid treatment has been shown to improve survival in these patients. In this article, a lamellar ichthyosis case is presented of an infant who had the symptoms at birth and was treated successfully with acitretin.Case Presentation: A term newborn infant presented after delivery. Physical examination showed that the skin on her outer mouth, neck, axillae, and inguinal fold areas had collodion membranes and peelings. On the third day of life, the skin all over her body became dry and seemed similar to parchment paper, with peeling in some areas, as well as ectropion and eclabium development. After her daily bath, liquid Vaseline was applied all over her body, but it did not provide enough benefits. The infant was started on acitretin treatment. On the 14th day of treatment, the skin appeared nearly normal. On the 28th day of life, the infant was discharged.Conclusions: Early oral retinoid treatment facilitates increased quality of life improves survival rates for ichthyosis patients.

The pat ient was a female neonate bor n prematurely on 36 weeks of gestation by normal vaginal delivery. During pregnancy, mother had no history of fever, drug use or x-ray exposure.The mother was gravida 2 and had no history of abortion. Her first baby was well.

Background: The name ichthyosis is derived from the Greek ikhthus meaning "fish" and refers to the similarity in appearance of the skin to fish scale. The ichthyoses are a heterogeneous group of disorders. There are few studies about the oral manifestations of these disorders. But early reports of ichthyosis in the Indian and Chinese literature date back to several hundred years.Case Presentation: Oral manifestations of the 14-year-old female patient with ichthyosis are presented. Physical examination revealed thick, brownish scales covering the entire body surface including all larger body flexures and corneae. She had short and dry hair. There were no nail abnormalities and hearing loss.Conclusion: We consider that this patient represents a new manifestation of lamellar ichthyosis disease, because congenitally teeth missing and cephalometric analysis measurements have not been reported before.

Introduction: Ichthyosis is an epidermal disruption that increases insensible water loss. Hypernatremic dehydration is a consequence of skin disruption. This study reviewed the treatment of hypernatremic dehydration in patients with ichthyosis comparing to patients with intact skin. Case Presentation: We studied five neonates with hypernatremia, including three ichthyosis cases and two normal-skin neonates. This case-series study showed that the sodium correction rate is slower in infants with ichthyosis than in infants with normal skin. The first and second neonates needed less sodium than fluid intake than normal skin infants, although fluid requirement was lower in the third ichthyosis infant than in others due to less skin disruption in this infant. Conclusions: Fluid therapy in hypernatremic dehydration in ichthyosis patients is different from neonates with intact skin because of excessive insensible water loss in these patients. It may be needed to give more fluid and less sodium depending on the degree of skin disruption, which may not be determined by physical examination.