Document Detail


Protean manifestations of lipoid proteinosis in a 16-year-old boy.
MedLine Citation:
PMID:  10671967     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We report a 16-year-old Japanese male with lipoid proteinosis showing various skin manifestations. The patient was born to nonconsanguineous parents and none of his relatives was similarly affected. The patient suffered from a hoarse voice and refractory temporal epilepsy from early childhood. Computed tomography scanning of the brain showed bilateral calcification in the temporal lobes, a characteristic feature of lipoid proteinosis. On physical examination, various skin manifestations, including papules and haemorrhagic blisters, acne-like scars at sites of minor trauma or friction, and beads of small papules along the free margins of the eyelids were noted. A skin biopsy showed deposits of homogeneous hyaline-like material, positive on periodic acid-Schiff staining, throughout the dermis, particularly around small blood vessels. It is noteworthy that a range of characteristic skin lesions can be present in a patient with lipoid proteinosis even with mild systemic involvement.
Authors:
T Nagasaka; M Tanaka; D Ito; K Tanaka; H Shimizu
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Clinical and experimental dermatology     Volume:  25     ISSN:  0307-6938     ISO Abbreviation:  Clin. Exp. Dermatol.     Publication Date:  2000 Jan 
Date Detail:
Created Date:  2000-05-17     Completed Date:  2000-05-17     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  7606847     Medline TA:  Clin Exp Dermatol     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  30-2     Citation Subset:  IM    
Affiliation:
Departments of Dermatology; Neurology, Keio University School of Medicine, Tokyo, Japan. tnaga@kyorin-ua.c.jp
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Biopsy / methods
Cicatrix
Hoarseness / complications
Humans
Lipoid Proteinosis of Urbach and Wiethe / complications,  pathology*
Male

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