Ichthyosis prematurity syndrome (IPS) is a dermatological disease with known genetic causes. This syndrome is a rare subcategory of autosomal recessive  congenital ichthyosis (ARCI). It is associated with complications in the mid-trimester of a pregnancy leading to premature births. Although most prevalent in individuals of Scandinavian origin, there have also been scattered cases in people of Japanese, Italian and Indian ethnicity. This disorder is also referred to as ichthyosis congenital type IV. Signs and symptoms
The symptoms associated with the disorder are often confused for other dermatological disorders. The symptoms below are ones specifically associated with IPS. Premature birth
Pregnancies that have a foetus affected with this syndrome are complicated because of polyhydramnion. Complications arise because of opaque amnionic fluid resulting from the shedding of skin. As a result, ultrasounds are difficult to conduct. Triggered by the harsh environment in the uterus, delivery results around 30– 34 weeks of gestation (pregnancy) and the baby is born in prematurely. Thick caseous layer of skin
A white layer of thick scaly substance covers the surface of the skin of the infant. This is characterized by aggregating membranes in the upper layer of skin on epidermal cells. Desquamating skin
Red endemic skin as well as spongy and desquamating (peeling) skin are consistent with this syndrome. Respiratory problems
After premature delivery, the baby often has neonatal asphyxia from breathing amnionic debris mainly composed of shedding skin cells. Neonatal refers to the very young infant and amniotic fluid refers to the liquid environment the baby is bathed in inside the uterus.
Prognosis
There are no life-threatening complications after the perinatal period (around the time of birth) and the skin conditions persist but to a lesser degree of severity. Individuals have a favourable prognosis as symptoms can be managed and past the infancy stage are not life-threatening. The red skin edema improves after a three-week period but the ichthyosis scaling persists. Asthma has been recorded in some cases later on in the individuals life and sign of atopic dermatitis persist, follicular hyperkeratosis and small amounts of scaling at the scalp that goes on into adulthood but otherwise the individual continues a healthy life. Epidemiology
Frequencies of this disease are the greatest in Norway with a few Finnish cases have also having been noted to date. Some cases have been found in other ethnicities such as in people of Indian or Japanese descent as well as a north Italian family. These cases are scattered and there are potentially more under reported cases as this disease is often under diagnosed for other cutaneous diseases. It is most prevalent in a defined region in the middle of Norway and Sweden with a heterozygote carrier frequency of 1 in 50. References


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