Document Detail


Cowden's disease: analysis of fourteen new cases.
MedLine Citation:
PMID:  6512057     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Fourteen new patients with Cowden's disease from the Netherlands were analyzed and compared with the sixty-nine previously reported patients. The full clinical spectrum of the disease can now be fairly well delineated. Mucocutaneous lesions, especially facial trichilemmomas and other follicular malformations, acral keratoses, and oral papillomas, remain the most constant findings. The incidence of each of these mucocutaneous findings was slightly higher than reported previously, probably reflecting increasing experience with this condition. Also our patients tended to have more extensive acral keratoses and perhaps less extensive facial lesions. In one family there was an extremely high incidence of lipomas. Breast cancer was seen in three of our twelve female patients, reflecting the trend toward a lower incidence of breast cancer in the recent literature. Gastrointestinal polyps in one family were much more common than previously reported, occurring in five of seven patients. Thyroid disease was found in about two thirds of the patients and abnormalities of the female reproductive system in slightly more than half of the patients, both incidences being approximately the same as in the literature. With increased awareness of the condition it is likely that many more cases will be recognized in the future.
Authors:
T M Starink
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of the American Academy of Dermatology     Volume:  11     ISSN:  0190-9622     ISO Abbreviation:  J. Am. Acad. Dermatol.     Publication Date:  1984 Dec 
Date Detail:
Created Date:  1985-02-05     Completed Date:  1985-02-05     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  7907132     Medline TA:  J Am Acad Dermatol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  1127-41     Citation Subset:  IM    
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MeSH Terms
Descriptor/Qualifier:
Adult
Aged
Bone and Bones / abnormalities
Breast Neoplasms / genetics
Congenital Abnormalities / genetics
Eye Diseases / genetics
Facial Neoplasms / genetics
Female
Gastrointestinal Neoplasms / genetics
Genes, Dominant*
Hamartoma / genetics*
Humans
Male
Middle Aged
Mouth Neoplasms / genetics
Neoplasms, Multiple Primary / genetics*
Netherlands
Papilloma / genetics
Skin Neoplasms / genetics*
Syndrome
Thyroid Neoplasms / genetics

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


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