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Incontinentia Pigmenti, a rare genodermatosis - Two case reports



Incontinentia Pigmenti (IP) or Bloch-Sulzberger syndrome is an uncommon genodermatosis that usually  occurs in females and characterized by cutaneous,  neurologic, ophthalmologic and dental manifestations. It is an X linked dominant neurocutaneous syndrome and is lethal in males. We report 2 cases of incontinentia pigmenti- one in a 19 year old female (case 1) with skin, dental and neurological manifestations and another in a 2 year old female child (case 2) with skin,ophthalmologic and neurological manifestations .Our patient (case 1) has demonstrated a rare clinical association of incontinentia pigmenti with hypoplasia of the breast.


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