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Congenital Ichtyosis


Lamellar ichtyosis, epidermolytic (bullous) ichtyosis, ichtyosis (congenital).


ICD-10: Q80.9
ORPHA: 79354

Estimated occurrence

2-4:100,000 live births


A change in one of the genes (15 known so far) that are required for the epidermis to form a horny layer. Autosomal recessive inheritance is the most common.

General symptoms

Congenital ichtyosis is characterised by skin symptoms. The skin can be scaly, red, dry, thickened, itchy, and covered in sores as the skin’s protective function is affected. The following main groups also have congenital skin symptoms:

  • lamellar ichtyosis
  • erythrodermic ichtyosis
  • pleomorphic ichtyosis
  • epidermolytic (bullous) ichtyosis.

The degree of flakiness and redness varies between the groups.

Orofacial/odontological symptoms

Congenital ichtyosis in and of itself has no effect on teeth, occlusion or oral motor function, but if facial skin is tight, it may be harder to open the mouth.

Orofacial/odontological treatment

  • In connection with dental treatment, any difficulties opening the mouth should be taken into consideration.


  • The rare disease database of the Swedish National Board of Health and Welfare.
  • The MHC database - The Mun-H-Center database on oral health and orofacial function in rare diseases.
  • The Documentation from the Ågrenska national competence centre for rare diseases.
Updated: 2017-09-12 13:55