The Indian Journal of Pediatrics

, Volume 80, Issue 11, pp 968–971

Incontinentia Pigmenti Presenting as Encephalopathy


    • Indira Gandhi Institute of Child Health
  • Chandra Mouly
    • Indira Gandhi Institute of Child Health
  • Asthik Biswas
    • Indira Gandhi Institute of Child Health
  • Shivananda Shivananda
    • Indira Gandhi Institute of Child Health
Clinical Brief

DOI: 10.1007/s12098-012-0916-4

Cite this article as:
K. N. Gowda, V., Mouly, C., Biswas, A. et al. Indian J Pediatr (2013) 80: 968. doi:10.1007/s12098-012-0916-4


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 pigmentiEncephalopathySkin rashesSeizures

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© Dr. K C Chaudhuri Foundation 2012