Document Detail

Incontinentia pigmenti.
MedLine Citation:
PMID:  20551538     Owner:  NLM     Status:  MEDLINE    
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.
Mohammad Hosein Kalantar Motamedi; Ali Lotfi; Taghi Azizi; Mohammad Moshref; Sareh Farhadi
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Indian journal of pathology & microbiology     Volume:  53     ISSN:  0974-5130     ISO Abbreviation:  Indian J Pathol Microbiol     Publication Date:    2010 Apr-Jun
Date Detail:
Created Date:  2010-06-16     Completed Date:  2010-09-16     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7605904     Medline TA:  Indian J Pathol Microbiol     Country:  India    
Other Details:
Languages:  eng     Pagination:  302-4     Citation Subset:  IM    
Department of Pathology, Trauma Research Center, Baqiyatallah University of Medical Sciences, and Attending Surgeon, Azad University of Medical Sciences, Tehran, Iran.
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MeSH Terms
Incontinentia Pigmenti / diagnosis*,  pathology*
Maxilla / pathology
Nails, Malformed / pathology
Skin / pathology
Toe Phalanges / pathology
Tooth / pathology

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