Article
Buschke-Ollendorff syndrome associated with elevated elastin production by affected skin fibroblasts in culture.
The Journal of investigative dermatology - 1 Aug 1992
Giro M G, Duvic M, Smith L T, Kennedy R, Rapini R, Arnett F C, Davidson J M
Abstract excerpt
Buschke-Ollendorff syndrome (BOS; McKusick 16670) is an autosomal dominant connective-tissue disorder characterized by uneven osseous formation in bone (osteopoikilosis) and fibrous skin papules (dermatofibrosis lenticularis disseminata). We describe two patients in whom BOS occurred in an autoso...
Topics
- Blotting, Southern
- Cells, Cultured
- Connective Tissue Diseases
- Elastin
- Female
- Fibroblast Growth Factor 2
- Fibroblasts
- Humans
- Male
- Microscopy, Electron
- Middle Aged
- Nevus
- Osteopoikilosis
- Pedigree
- Phenotype
- RNA, Messenger
- Skin
- Skin Neoplasms