Buschke-Ollendorff syndrome

  • Joint Bone Spine. 2008 Mar;75(2):212-4. doi: 10.1016/j.jbspin.2007.04.027.
Hasna Hassikou  1 ,  Fatima Tabache ,  Soumaya Safi ,  Mohamed Baaj ,  Larbi Hadri
Affiliations
  • 1. Service de Médecine Interne, Hôpital militaire Moulay Ismail, Meknès, Morocco.
PMID: 18313966 DOI: 10.1016/j.jbspin.2007.04.027
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.