Buschke-Ollendorff syndrome.
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.
Hassikou
Hasna
H
Service de Médecine Interne, Hôpital militaire Moulay Ismail, Meknès, Morocco.
Tabache
Fatima
F
Safi
Soumaya
S
Baaj
Mohamed
M
Hadri
Larbi
L
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
D004268:DNA-Binding Proteins; C405329:LEMD3 protein, human; D008565:Membrane Proteins; D009687:Nuclear Proteins; D003094:Collagen