Hypomelanosis of Ito appears to be the third most common neurocutaneous disease after neurofibro-matosis and tuberous sclerosis. The bizarre, patterned, hypopigmented macules usually appear during the first year of life or at birth in sharply demarcated whorls, patches or streaks that follow the lines of Blaschko. The lesions appear as a negative image of incontinentia pigmenti. Multiple extracutaneous abnormalities involving the nervous system, eyes, musculoskeletal, hair, head, face, dental, cardiovascular, genito-urinary system and other organ anomalies can occur in most of the patients. We report an interesting case of Hypomelanosis of Ito with tessellated fundus and polymicrogyria.

Fig.1 A child with Hypomelanosis of Ito showing multiple whorled like hypopigmented lesions present over abdomen, chest and back.

A 4 years old male child was brought with recurrent episodes of generalized tonic clonic seizures since 8 months of age. There was global developmental delay, hypotonia and mental retardation. On examination, multiple whorled like hypopigmented macules were present over abdomen, chest, back and both lower limbs arranged along the lines of Blaschko since birth (Fig. 1). The palms, soles and mucous membrane were spared. The fundus showed pale disc, tortous vessels around the disc, radial patchy streaks of hypopigmentation and tessellated background (Fig. 2). EEG showed generalized epileptiform discharges. CT and MRI scan brain showed hemispheric asymmetry with atrophy of right cerebral hemisphere and ballooning of right lateral ventricle. MRI brain demonstrated polymicrogyria at right perisylvian and parietal region, thinning of corpus callosum and periventricular hyperintense signal.

Fig.2 Fundus showing pale disc, tortous vessels around the disc, radial patchy streaks of hypopig-mentation and tessellated background.

Classical tessellated fundus in Hypomelanosis of Ito has been reported only in two children in literature(1). Also, there are very few reports about polymicrogyria in Hypomelanosis of Ito(2,3).

Prof ML Vasanthakumari, Dr K Mathiarasan, Dr M Balasubramaniam, Dr S Nataraja Rathinam and Dr V Suba, for their valuable suggestions and kind cooperation to evaluate this case.