KUMJ | VOL. 11 | NO. 1 | ISSUE 41 | JAN-MAR, 2013
Incontinentia Pigmenti
BS G, Pai GS, Pai AH, Vinekar AS, Pai HS, Noronha T, Fernandes MS
Abstract: Incontinentia pigmenti is an X- linked dominant condition characterized by
cutaneous lesions associated with developmental defects of the eye, skeletal
system and central nervous system. We report a case of incontinentia pigmenti
in a 30 day old female infant who presented to us with skin eruptions over
bilateral upper limbs, lower limbs and trunk since birth. She had linear verrucous
plaques and vesicles distributed along the Blaschko’s lines in addition to macular
hyperpigmentation in a linear and whorled pattern involving the concerned
areas. On ophthalmological examination, proliferative retinopathy in the right
eye was noted.
Keyword : Incontinentia pigmenti, proliferative vitreoretinopathy