Clinical Brief

The Indian Journal of Pediatrics

, Volume 80, Issue 11, pp 968-971

First online:

Incontinentia Pigmenti Presenting as Encephalopathy

  • Vykuntaraju K. N. GowdaAffiliated withIndira Gandhi Institute of Child Health Email author 
  • , Chandra MoulyAffiliated withIndira Gandhi Institute of Child Health
  • , Asthik BiswasAffiliated withIndira Gandhi Institute of Child Health
  • , Shivananda ShivanandaAffiliated withIndira Gandhi Institute of Child Health

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Incontinentia Pigmenti is a rare neurocutaneous disorder that may present with neurological symptoms in early infancy in addition to characteristic skin rashes. We report a two-mo-old girl presenting with a rash and encephalopathy. Magnetic resonance imaging of brain showed hemorrhagic infarcts. First we thought baby was suffering from a skin infection with meningo-encephalitis and later with development of characteristic skin lesions, neuroimaging and skin biopsy changes we reconsidered our diagnosis as Incontinentia pigmenti.


Incontinentia pigmenti Encephalopathy Skin rashes Seizures