U.S. flag

An official website of the United States government


Send to:

Choose Destination

Search results

Items: 2


Dermatofibrosis lenticularis disseminata

Buschke-Ollendorff syndrome (BOS) is an autosomal dominant connective tissue disorder manifest by multiple subcutaneous nevi or nodules. They may be either elastin-rich (elastoma) or collagen-rich (dermatofibrosis lenticularis disseminata) on histologic examination. The lesions are usually nontender and firm. Affected individuals also have osteopoikilosis (OPK), literally meaning 'spotted bones,' which are osteosclerotic foci that occur in the epiphyses and metaphyses of long bones, wrist, foot, ankle, pelvis, and scapula. Some individuals have both skin and bone manifestations, whereas others may lack skin or bone manifestations. Some individuals may also have melorheostosis (155950), which is characterized by 'flowing' hyperostosis of the cortex of tubular bones. Most reported cases of BOS and OPK are benign, and the bone lesions are found incidentally, although some patients may have joint pain (reviews by Hellemans et al., 2004 and Zhang et al., 2009). [from OMIM]

MedGen UID:
Concept ID:
Disease or Syndrome

Connective tissue nevi

Connective tissue nevi are hamartomas in which one or several components of the dermis is altered. [from HPO]

MedGen UID:
Concept ID:
Neoplastic Process

Supplemental Content

Find related data

Search details

See more...

Recent activity