Document Detail


Coexistence of two neurocutaneous syndromes: tuberous sclerosis and hypomelanosis of Ito.
MedLine Citation:
PMID:  17314448     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Tuberous sclerosis complex (TSC) and hypomelanosis of Ito (HI) are two uncommon neurocutaneous syndromes and their coexistence is extremely rare. An epileptic child presented with progressively increasing multiple hypopigmented macules arranged in a linear and whorled pattern along the lines of Blaschko over the trunk and limbs, characteristic of HI. He also had facial angiofibromas, ash-leaf and confetti macules and shagreen patches. Magnetic resonance imaging of the brain showed cortical tubers and subependymal nodules; which are diagnostic of TSC. The TSC defining loci have been mapped to Chromosome 9q34 (TSC1) and 16 pl3.3 (TSC2). There is no common genetic background for HI, but mosaicism of 9q33 locus has been documented. As per our knowledge, this is the second case of association of TSC with HI in a four-year-old child.
Authors:
K Muhammed; Jenny Mathew
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Indian journal of dermatology, venereology and leprology     Volume:  73     ISSN:  0973-3922     ISO Abbreviation:  Indian J Dermatol Venereol Leprol     Publication Date:    2007 Jan-Feb
Date Detail:
Created Date:  2007-02-22     Completed Date:  2007-03-22     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7701852     Medline TA:  Indian J Dermatol Venereol Leprol     Country:  India    
Other Details:
Languages:  eng     Pagination:  43-5     Citation Subset:  IM    
Affiliation:
Department of Dermatology and Venereology, Medical College, Kozhikode, Kerala, India. drmuhammedk@rediffmail.com
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MeSH Terms
Descriptor/Qualifier:
Angiofibroma / complications
Brain / pathology
Child, Preschool
Epilepsy / complications
Facial Neoplasms / complications
Humans
Hypopigmentation / complications*,  diagnosis
Magnetic Resonance Imaging
Male
Mental Retardation / complications
Tuberous Sclerosis / complications*,  diagnosis

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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