Ichthyosis in the newborn

Abstract

The ichthyoses encompass a variety of genetic disorders marked by abnormal epidermal differentiation. The neonatal period is critical for patients with ichthyosis because of the risk for significant associated morbidity and mortality, with the majority of complications arising as a result of impaired barrier function. This article reviews presentations of ichthyosis in the neonate, outlines risks and complications, and provides strategies for management.

Ictiosis arlequín, una genodermatosis devastadora

“…4 Sin embargo, la supervivencia está directamente relacionada con el tipo de mutaciones, es así como las víctimas con la mutación heterocigótica compuesta sobreviven más que aquellas con la mutación homocigoto 8 , además, con el cuidado intensivo neonatal avanzado en combinación con el manejo apropiado de la piel, muchos niños ahora sobreviven. 9 …”

Collodion Baby: Case Report

“…Patients suffering from severe forms of Ichthyosis benefit from a humid environment and electrolyte balance control [7]. Intravascular hydration through the peripheral pathway is not recommended due to the characteristic lesions of these patients, it is usually recommended by umbilical vascular catheterization, which is less related to infections [3]. Topical treatments such as urea, alpha hydroxy acids, mainly lactic acid and propylene glycol, vegetable oils such as petroleum jelly and topical retinoid are used [2].…”

“…Topical treatments such as urea, alpha hydroxy acids, mainly lactic acid and propylene glycol, vegetable oils such as petroleum jelly and topical retinoid are used [2]. The solutions that are contraindicated in these patients are silver sulfate which is related to agranulocytosis and baths with chlorhexidine because of its toxic potential [3].…”

“…In turn, it may present with acquired disorders. They are distinguished by their mode of inheritance, clinical characteristics, associated defects and histological findings [1][2][3][4][5][6]. Several classifications of this pathology are known, Traupe proposed to divide them into vulgar, when the clinic is incipient at birth and congenital when they are marked at the time of birth [2].…”

“…The collodion membrane breaks off between the 1 st and 4 th week of life, evidencing the definitive phenotype. 60% of the cases correspond to the initial expression of Congenital Ichthyosiform Erythroderma or Lamellar Ichthyosis, however, 10% will have normal skin after resolution of the collodion membrane [3,4]. Other presentations include Ichthyosis Harlequin, Linked to Chromosome X, Erythroderma Ichthyosiform congenital blister and Nethertan Syndrome.…”