Hypomelanosis of Ito Clinical Presentation

Updated: May 07, 2018
  • Author: Manuel Valdebran, MD; Chief Editor: Dirk M Elston, MD  more...
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Patients with hypomelanosis of Ito usually seek care from a dermatologist, pediatrician, or neurologist by the time they are aged 2 years.

The hypopigmentation is not preceded by vesicular or verrucous lesions that are usually seen in incontinentia pigmenti.

A family history of hypomelanosis of Ito is rare.

The patient or parents should be asked about the following:

  • Seizures

  • Mental retardation

  • Developmental delay

  • Deafness

  • Visual problems

  • Headache

  • Tooth or mouth problems

The most common reported associations are congenital abnormalities, mental retardation, and seizures. [9] Cerebral malformations may occur, and visual impairment may be cortical in nature. [10, 11] Glomerulocystic kidney disease has also been reported. [12]


Physical Examination

Small 0.5- to 1-cm hypopigmented macules coalesce to form reticulated patches along the lines of Blaschko, usually stopping at the midline. They are most often seen on the trunk and limbs. The macules/patches cover more than two dermatomes and are often on both sides of the body. A Wood lamp enhances the pattern, especially in white patients. [8] See the images below.

Hypomelanosis of Ito on the arm. Hypomelanosis of Ito on the arm.
Hypomelanosis of Ito highlighted with a Wood lamp. Hypomelanosis of Ito highlighted with a Wood lamp.
Hypomelanosis of Ito on the torso. Hypomelanosis of Ito on the torso.

Careful full body examination is needed to detect dysmorphism, including the following:

  • Cleft palate

  • Hemihypertrophy

  • Limb, hand, and/or foot abnormalities

  • Nail abnormalities

  • Hypotonia

  • Teeth abnormalities

  • Hair anomalies

  • Face and/or skull anomalies

Neurologic examination is essential to evaluate neural tumors, seizures, and psychomotor delay.



Complications may occur as a result of associated abnormalities.