| CASE REPORT |
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| Year : 2010 | Volume
: 53
| Issue : 2 | Page : 302-304 |
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Incontinentia pigmenti
Mohammad Hosein Kalantar Motamedi1, Ali Lotfi2, Taghi Azizi3, Mohammad Moshref2, Sareh Farhadi2
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
 Source of Support: None, Conflict of Interest: None  | Check |
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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