Buschke–Ollendorff syndrome - 19/04/08
, Fatima Tabache, Soumaya Safi, Mohamed Baaj, Larbi Hadri| pagine | 3 |
| Iconografia | 2 |
| Video | 0 |
| Altro | 0 |
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.
Il testo completo di questo articolo è disponibile in PDF.Keywords : Buschke–Ollendorff syndrome, Osteopoikilosis
Mappa
Vol 75 - N° 2
P. 212-214 - marzo 2008 Ritorno al numeroBenvenuto su EM|consulte, il riferimento dei professionisti della salute.
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