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Case Studies | Pediatrics | India | Volume 11 Issue 6, June 2022 | Rating: 4.1 / 10

Rare Case Report on Incontinentia Pigmenti

Dr. Tinkal Patel | Dr. Devavrat Bhide | Dr. Panna Patel | Dr. Apoorva Shah

Abstract: Incognentiapigmenti is a neurocutaneous syndrome. It is a x - linked dominant disorder. It is a rare, inheritable, multisystem ectodermal disorder. Pathogenic IKBKG gene or 47, XXY karyotyping seen in female X - inactivation (45, X) seen. Most commonly involved organ is skin. Other systems involved are CNS, ocular, dental and hair.

Keywords: Incontinentia Pigmenti, neurocutaneous syndrome

Edition: Volume 11 Issue 6, June 2022,

Pages: 1559 - 1560