Preferred Name |
Buschke-Ollendorff syndrome |
|
Synonyms |
BOS |
|
Definitions |
(BOS) - A disease characterized by osteopoikilosis and disseminated connective- tissue nevi. Osteopoikilosis is a skeletal dysplasia characterized by a symmetric but unequal distribution of multiple hyperostotic areas in different parts of the skeleton. Elastic-type nevi (juvenile elastoma) and collagen-type nevi (dermatofibrosis lenticularis disseminata) have been described in BOS. Skin or bony lesions can be absent in some family members, whereas other relatives may have both. |
|
ID |
http://identifiers.org/omim/166700 |
|
altLabel |
BOS |
|
definition |
(BOS) - A disease characterized by osteopoikilosis and disseminated connective- tissue nevi. Osteopoikilosis is a skeletal dysplasia characterized by a symmetric but unequal distribution of multiple hyperostotic areas in different parts of the skeleton. Elastic-type nevi (juvenile elastoma) and collagen-type nevi (dermatofibrosis lenticularis disseminata) have been described in BOS. Skin or bony lesions can be absent in some family members, whereas other relatives may have both. |
|
id |
OMIM:166700 |
|
notation |
OMIM:166700 |
|
prefLabel |
Buschke-Ollendorff syndrome |
|
subClassOf |