[Iconography of Buschke Ollendorff syndrome: x ray computed tomography and nuclear magnetic resonance of osteopoikilosis].
The authors report a case of Buschke-Ollendorff syndrome in a 66-year old woman who had been admitted for polymyalgia rheumatica. Dermatological examination showed innumerable small ivory papullae bilaterally and symmetrically distributed over the back, the shoulders, the flexor aspect of the elbows, the wrists and the thighs. Biopsy of a papulla displayed hypertrophy of the elastic tissue condensed in the deep dermis. Electron microscopy showed dense collagen bundles together with an absence of microfibillae and of anchoring fibres in the superficial dermis. Standard radiology, computed tomography (CT) and nuclear magnetic resonance (NMR) disclosed small areas of "stippled" bone densification in the lower middle part of the femoral heads, leading to a diagnosis of osteopoikilosis. Buschke-Ollendorff syndrome was described in 1928 as "dermatofibrosis lenticularis disseminata" associated with a bone-condensing disease called "osteopoikilosis". The condition is transmitted as an autosomal dominant trait. Light and electron microscopy of the skin show densification of collagen structures and elastic fibres that are very numerous and of abnormal structure. Osteopoikilosis may present under two different forms, punctate (Albers-Schomberg syndrome) or striated, corresponding anatomically to areas of compact bone. CT and NMR provide detailed information on the location and structure of bone condensation area.
Dahan
S
S
Unité de Dermatologie, CHU Toulouse Rangueil.
Bonafé
J L
JL
Laroche
M
M
Rousseau
H
H
Railhac
J J
JJ
D000368:Aged; D003937:Diagnosis, Differential; D005260:Female; D005350:Fibroma / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D010023:Osteopoikilosis / Q000175:diagnosis* / Q000000981:diagnostic imaging; D010026:Osteosclerosis / Q000175:diagnosis* / Q000235:genetics; D011111:Polymyalgia Rheumatica / Q000175:diagnosis; D012871:Skin Diseases / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology; D013577:Syndrome; D014057:Tomography, X-Ray Computed
Iconographie du syndrome de Buschke-Ollendorff: étude tomodensitométrique et en résonance magnétique nucléaire de l'ostéopoecilie.