Ito hypomelanosis

What is Ito hypomelanosis?

Ito's hypomelanosis is part of a rare genetic neurocutaneous syndrome. It is characterized by:

• Striped, irregular, spiral or linear hypopigmented macules that occurs in any part of the body
• Palms, scalp and soles of the feet are generally not affected
• The patterns that revolve around the trunk and the linear patterns for the legs and arms are called Blaschko lines.
• Lesions first appear as small 0.5-1 cm white or hypopigmented macules that fuse to form larger patches.
• Macules cover more than two dermatomes and are often on both sides of the body.
• The patches are not symmetrical

Ito's hypomelanosis is also known as "incontinentia pigmenti achromians".

Abnormalities associated with Ito hypomelanosis

Associated abnormalities are believed to occur in 30-50% of patients with cutaneous Injuries These include:

• Seizures
• Mental retardation
• Hearing abnormalities
• Tooth or mouth problems
• Visual problems
• Orthopedic problems

What is the cause of Ito hypomelanosis?

Ito hypomelanosis is believed to be due to chromosomal mosaicism and sporadic mutations. It is not an inherited disorder since the chromosomal defect occurs after conception. I specify gene(s) is most likely a balanced translocation from Xp21.2.

How is the diagnosis of hypomelanosis of Ito done?

Ito hypomelanosis is present at birth and is usually detected by a dermatologist, pediatrician or neurologist during the first or second year of life. Complete history taking and physical examination with attention to neurological and ophthalmological The findings are necessary to detect associated abnormalities.

Although recent research has estimated that 30-50% of Ito hypomelanosis patients have associated abnormalities, some studies report associated neurological abnormality rates as high as 75-94%.

What is the treatment for Ito hypomelanosis?

The cutaneous effects of Ito hypomelanosis do not require treatment. In many cases, the skin can develop. pigment over time and mix well with normal skin. Patients who are aware of their appearance can use cosmetic camouflage.

Associated abnormalities require appropriate medical treatment.