Ichthyosis in children

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Philippe Gloaguen
@philippegloaguen
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Ichthyoses are a group of genetic skin diseases, characterized by a strong tendency to generalized skin peeling (in fact the word derives from the Greek Iχτυς = fish)



There are pure cutaneous forms, which are called non-syndromes ichthyosis, and forms that are associated with disorders affecting other organs which are called syndromic ichthyosis.

With the advice of Prof. Maya El Hachem, head of the Dermatology Operating Unit at the Bambino Gesù Children's Hospital in the city, we have developed a mini guide.



How they manifest





The most common forms are common ichthyosis, in particular ichthyosis vulgaris and X-linked recessive.

  • Vulgar ichthyosis it is not present at birth, but manifests itself over the years with thin and medium-sized scales in the limb area, often associated with atopic dermatitis;

  • l’Ittiosi X-linked recessive (derived from healthy carrier parents) instead manifests itself already in the first month of life, only in the male sex (since, as the name implies, it is linked to the X chromosome).

What are the causes

At the base there is a genetic defect of the corneification, that is the process of formation and detachment of the cells of the stratum corneum (the most superficial layer of the skin).

How to cure

To date, there are no therapies capable of resolving the molecular and enzymatic defect that causes ichthyosis, but the treatments are symptomatic and aimed at restoring the functions of the skin barrier, through creams and ointments with keratolytic action, emollient creams, calcineurin inhibitors, plus any systemic treatments based on retinoids, to be used in selected cases and under strict dermatological control.

Due to the aesthetic discomfort that this pathology entails, especially in school and adolescent age, psychological support is often necessary in order to ensure adequate school placement and a good quality of relational life.

To know more: Unione Del Paesena Ittiosi http://www.comtiosi.com/index.asp

Do you want to know everything about dermatological diseases?

Read our special on CHILDREN'S DERMATITIS

TAG:
  • ittiosi
  • first year
  • dermatitis
  • care
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