
LAMELLAR ICHTYOSIS ( AND ICHTYOSIS TYPE "ARLEQUIN")
Ichtyoses form a heterogeneous group of dermatological diseases which are
characterized by extremely dry skin, with increased thickness of the outer layer
of the epidermis (the stratum corneum) and build-up of dead skin, looking like
fish scales. Many forms of ichtyoses have been described, but their
classification is sometimes difficult, because of their large clinical and
genetic heterogeneity. Lamellar ichtyosis is one form of congenital ichtyosis.
This rare autosomal (1) recessive (2) disease affects 1 new-born baby in 300 000.
Is characterized by:
- a dry skin disorder with frequent and abundant exfoliation
- an anomaly of the eyelids
- impaired movement development, (standing, balance, etc).
This pathology requires:
- frequent skin care (medicinal baths and creams) and eye treatment,
- a specific oral treatment,
- a regular medical follow-up,
- physiotherapy (manipulation, splints).
The patient fears all that drains the skin and the mucous membranes:
- light,
- heat,
- cold,
- hard water.
It should be emphasised to all those who are in contact with a person with ichtyosis that the disease is not contagious.
1). It affects both men and women.
2). The disease is a result of a gene mutation, present in both parents