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Introduction: Incontinentia pigmenti (IP) or Bloch- Sulzberger syndrome is a rare X linked dominant
genodermatosis with multiorgan involvement due to a mutation in NEMO gene. It mostly affects females as it could
be lethal in males. Ectodermal in origin, this disorder gives rise to ocular, dental, skin and neurological
manifestations. Landy and Donnai have introduced criteria for diagnosis of IP in 1993.
Case Report: We report a 2-year-old girl (index case) and her mother who fulfil the above criteria for IP. The child
has had all four stages of cutaneous manifestations since birth while the mother had three. The girl also had
characteristic dental and hair manifestations. She is developmentally and neurologically normal. The mother has
ODhthalmological, nail and hair changes of IP and suffers from seizures. Her dentition is normal. Maternal family
y is insignificant. Index case is the only child and there were no miscarriages in the mother.
. Hussion: Integratedmulti-disciplinary management including genetic counseling arranged for both patients. This
is the first Sri Lankan publication on IP in two generations with classic clinical features.
