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Buschke-Ollendorff syndrome
Hasna Hassikou1, Fatima Tabache, Soumaya Safi
1Service de Médecine Interne, Hôpital militaire Moulay Ismail, Meknès, Morocco.
Unlabelled:
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.
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