Document Detail


Phacomatosis pigmentokeratotica associated with a suprasellar dermoid cyst and leg hypertrophy.
MedLine Citation:
PMID:  17868392     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Phacomatosis pigmentokeratotica (PP) is a mosaic disorder that represents a distinct epidermal naevus syndrome. Its defining features are an epidermal naevus that is usually of the sebaceous type and a speckled lentiginous naevus arranged in a chequerboard pattern. In addition, there are neurological, ophthalmological and skeletal abnormalities, including limb hemiatrophy with muscular weakness, ptosis, seizures and ipsilateral segmental hyperaesthesia and hyperhidrosis. We report a 44-year-old man with an extensive epidermal naevus and an ipsilateral speckled lentiginous naevus. He also had ipsilateral right leg hypertrophy and a suprasellar dermoid cyst with associated neurological abnormalities. We propose that this case represents an unusual example of PP.
Authors:
V Majmudar; A Loffeld; R Happle; A Salim
Publication Detail:
Type:  Case Reports; Journal Article     Date:  2007-09-14
Journal Detail:
Title:  Clinical and experimental dermatology     Volume:  32     ISSN:  0307-6938     ISO Abbreviation:  Clin. Exp. Dermatol.     Publication Date:  2007 Nov 
Date Detail:
Created Date:  2007-10-23     Completed Date:  2008-04-03     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7606847     Medline TA:  Clin Exp Dermatol     Country:  England    
Other Details:
Languages:  eng     Pagination:  690-2     Citation Subset:  IM    
Affiliation:
Department of Dermatology, Heart of England NHS Foundation Trust, Solihull, West Midlands, UK. vmajmudar@doctors.org.uk
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MeSH Terms
Descriptor/Qualifier:
Adult
Central Nervous System Cysts / diagnosis*
Dermoid Cyst / diagnosis*
Humans
Hypertrophy / diagnosis,  pathology
Leg / pathology*
Male
Neurocutaneous Syndromes / diagnosis*,  pathology

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


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