title: Buschke-Ollendorff syndrome.
abstract: Buschke-Ollendorff syndrome (BOS) is an autosomal dominant disorder characterized by elastin-rich hamartomas and osteopoikilosis.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Service de Médecine Interne, Hôpital militaire Moulay Ismail, Meknès, Morocco.
authors.forename: Hasna
authors.initials: H
authors.lastname: Hassikou
authors.forename: Fatima
authors.initials: F
authors.lastname: Tabache
authors.forename: Soumaya
authors.initials: S
authors.lastname: Safi
authors.forename: Mohamed
authors.initials: M
authors.lastname: Baaj
authors.forename: Larbi
authors.initials: L
authors.lastname: Hadri
mesh_terms: D000328:Adult; D001706:Biopsy; D025063:Chromosome Disorders / Q000175:diagnosis* / Q000235:genetics; D003094:Collagen / Q000378:metabolism; D004268:DNA-Binding Proteins; D004547:Elastic Tissue / Q000378:metabolism; D005260:Female; D006222:Hamartoma / Q000175:diagnosis* / Q000378:metabolism / Q000473:pathology; D006801:Humans; D008565:Membrane Proteins / Q000235:genetics; D009154:Mutation / Q000235:genetics; D009687:Nuclear Proteins / Q000235:genetics; D010023:Osteopoikilosis / Q000000981:diagnostic imaging*; D011859:Radiography; D012871:Skin Diseases / Q000175:diagnosis* / Q000378:metabolism / Q000473:pathology; D013577:Syndrome
chemical_list: D004268:DNA-Binding Proteins; C405329:LEMD3 protein, human; D008565:Membrane Proteins; D009687:Nuclear Proteins; D003094:Collagen