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 autosomal dominant inheritance pattern. The connective tissue of the skin lesions showed both collagen and elastin...
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