Hystrix-like ichthyosis–deafness syndrome

Hystrix-like ichthyosis–deafness syndrome (HIDS) is a rare genetic disorder characterized by severe skin scaling and hearing loss. It is also known as KID syndrome or Keratitis-Ichthyosis-Deafness syndrome.

Symptoms

The primary symptoms of HIDS include keratitis (inflammation of the cornea), ichthyosis (dry, scaly skin), and deafness. Other symptoms may include hair loss, dental abnormalities, and an increased risk of skin infections.

Causes

HIDS is caused by mutations in the GJB2 gene, which provides instructions for making a protein called connexin 26. This protein is found in the outer layer of the skin (the epidermis) and in the inner ear, where it plays a crucial role in maintaining the health and function of these tissues.

Diagnosis

Diagnosis of HIDS is based on the presence of characteristic symptoms and confirmed by genetic testing to identify mutations in the GJB2 gene.

Treatment

There is currently no cure for HIDS. Treatment is aimed at managing symptoms and may include the use of moisturizers and keratolytic agents to manage skin scaling, antibiotics to treat skin infections, and hearing aids to manage hearing loss.

See also

• Ichthyosis
• Deafness
• Keratitis
• GJB2

References

Hystrix-like ichthyosis–deafness syndrome Resources
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