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Joint Bone Spine. 2008 Mar;75(2):212-4. doi: 10.1016/j.jbspin.2007.04.027. Epub 2008 Mar 3.

Buschke-Ollendorff syndrome.

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1
Service de Médecine Interne, Hôpital militaire Moulay Ismail, Meknès, Morocco.

Abstract

Buschke-Ollendorff syndrome (BOS) is an autosomal dominant disorder characterized by elastin-rich hamartomas and osteopoikilosis.

CASE REPORT:

In a 21-year-old woman, osteopoikilosis led to the diagnosis of BOS. She had multiple, grouped, buff-colored papules over the thighs and trunk. There was no pain or pruritus associated with the skin lesions. Examination of a biopsy specimen from a papule showed thick uniform collagen fibers and normal numbers of broad interlacing elastic fibers.

DISCUSSION:

BOS is a rare disease that affects 1/20,000 population. The diagnosis rests on a thorough physical examination and careful examination of radiographs. BOS must be distinguished from other bone abnormalities such as sclerotic bone metastases, particularly when osteopoikilosis is the inaugural manifestation.

PMID:
18313966
DOI:
10.1016/j.jbspin.2007.04.027
[Indexed for MEDLINE]
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