ВРОЖДЕННЫЙ ИХТИОЗ (КОЛЛОДИЙНЫЙ ПЛОД) (КЛИНИЧЕСКОЕ НАБЛЮДЕНИЕ)

Abstract

Intensive research is currently underway on the treatment of congenital ichthyosis in newborns. The paper describes a case of lamellar congenital ichthyosis, manifested by the state of a "colloidal" fetus. Congenital ichthyosis is a serious disease in which the process manifests itself at the time of birth. At the same time, the skin of the child is covered with a thin, dry, yellowish-brown layer resembling a colloid; ectropion and eclabium are often noted. After some time, cracks appear, often deep, the film turns into large scales, a clinical picture of congenital ichthyosis is formed. Lamellar ichthyosis (LI) belongs to the group of autosomal recessive congenital ichthyosis of the skin and is caused by a significant impairment of the skin barrier function due to the inability of differentiated keratinocytes to synthesize and/or secrete lipids necessary for the formation of corneocytes and the extracellular matrix of the stratum corneum. For the first time the disease was described by E. Seligman (1841) under the name "lamellar desquamation of newborns". The term "lamellar ichthyosis" was proposed by L. Gross and L. Torok (1895). The disease is genetically heterogeneous, occurs with a frequency of 1:300,000 of the population, more often in closely related marriages. They are affected with equal frequency by males and females. The described clinical case vividly illustrates the features of the course of congenital ichthyosis, the effectiveness of timely therapy using modern skin care products in a short time without the use of hormonal drugs.