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CASE REPORT
Year : 2010  |  Volume : 53  |  Issue : 2  |  Page : 302-304

Incontinentia pigmenti


1 Department of Pathology, Trauma Research Center, Baqiyatallah University of Medical Sciences, and Attending Surgeon, Azad University of Medical Sciences, Tehran, Iran
2 Department of Pathology, Shahid Beheshti University of Medical Sciences, Tehran, Iran
3 Department of Pathology, Baqiyatallah University of Medical Sciences, Tehran, Iran

Correspondence Address:
Mohammad Hosein Kalantar Motamedi
Africa Expressway, Golestan St., Giti Blvd. No. 11 Tehran, 19667
Iran
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.64291

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Incontinentia pigmenti (IP) or Bloch-Sulzberger syndrome is a rare X-linked dominant genodermatosis related to the NF kappa B essential modulator (NEMO) gene with approximately 800 cases reported worldwide. It usually occurs in females characterized by cutaneous, skeletal, neurological, ocular and dental abnormalities as well as an increased risk of childhood malignancies. Herein, we report a case of IP in a 14-year-old girl emphasizing early diagnosis and adding to the current literature on the subject.


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