Ichthyosis Fruit

Fetal ichthyosis (i. fetus) is a rare congenital skin disease characterized by generalized hyperkeratosis. Synonyms: universal congenital hyperkeratosis, malignant keratoma.

With fetal ichthyosis, abnormal keratinization of the epidermis occurs, resulting in the child's skin becoming covered with thick scales. This leads to disruption of thermoregulation, sweating, respiratory and motor functions of the skin.

The disease is caused by mutations in genes encoding keratin proteins. Depending on the type of mutation, several forms of fetal ichthyosis are distinguished with varying severity.

Diagnosis is based on the characteristic clinical picture. Treatment is mainly symptomatic and is aimed at moisturizing and softening the skin. The prognosis for life is unfavorable; most children die in the first months of life. Prenatal diagnosis allows you to detect the disease in the early stages of pregnancy.

Softening of connective tissue and skin, leading to thickening (hyperkeratosis) and keratinization. As the fetus grows, the skin becomes rigid and tight (“skin like parchment,” “hide tanned in a tannery”), causing hypertension, shortness of breath, and suffocation. Onychomatosis is also associated with hypovitaminosis E, which causes peripheral circulatory disorders (Luzon syndrome). Rarely seen. It is an obligate sign of atopy in the fetus (see Differential diagnosis)[3].