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Genetic and Rare Diseases Information Center (GARD)

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Buschke Ollendorff syndrome

Other Names for this Disease
  • BOS
  • Dermatofibrosis lenticularis disseminata with osteopoikilosis
  • Dermatofibrosis, disseminated with osteopoikilosis
  • Dermatoosteopoikilosis
  • Osteopathia condensans disseminata
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What are the signs and symptoms of Buschke Ollendorff syndrome?

Buschke Ollendorff syndrome (BOS) is an association of connective tissue nevi and osteopoikilosis (small, round areas of increased bone density). The nevi are typically present on the trunk, in the sacrolumbar region (lower back and sacrum), and on the extremities (arms and legs). Occasionally, they may be on the head. The nevi are usually nontender and firm,[1] and are typically first noticeable as slightly elevated and flattened yellowish bumps, grouped together and forming plaques that may be several centimeters in diameter. The plaques are typically of irregular shape. They are usually numerous, painless, and develop over several years.[2] The osteopoikilosis typically occurs in the long bones, wrist, foot, ankle, pelvis, and scapula. They are harmless and usually found by chance when radiographs are taken for other purposes, although pain and limited joint mobility have been reported in some individuals.[3] In some individuals, only skin or bone manifestations may be present.[2]

Other signs and symptoms of BOS may include nasolacrimal duct obstruction, amblyopia ("lazy eye"), strabismus, benign lymphoid hyperplasia, hypopigmentation (abnormally light skin), and short stature. Congenital spinal stenosis (narrowing of the spine), disc herniation, clubfoot deformity, and nerve root compression may be present. Otosclerosis (abnormal growth of bone in the middle ear) with or without hearing loss may occur, but is rare.[2]
Last updated: 3/16/2011

  1. Cassandra L. Kniffin et al. BUSCHKE-OLLENDORFF SYNDROME; BOS. OMIM. June 2, 2010; Accessed 3/16/2011.
  2. Lukasz Matusiak, Grazyna Szybejko-Machaj, Jacek C Szepietowski. Dermatofibrosis Lenticularis (Buschke-Ollendorf Syndrome). eMedicine. August 16, 2010; Accessed 3/16/2011.
  3. G. Mortier. Buschke-Ollendorff syndrome. Orphanet. January 2009; Accessed 3/16/2011.