Berardinelli-Seip-Syndrom

Imaging

spectrum of abnormal subcutaneous and visceral fat distribution. A 23-year-old woman with congenital generalized lipodystrophy and fifth finger pain. Lytic lesion (arrowheads) was evident on (a) radiograph and (b) CT in the fifth middle phalange. c CT showed almost complete loss of fat tissue other than the palmar fat pad (arrow). MRI showed complete lack of bone marrow adipose tissue. Coronal (d) T2-weighted and (e) STIR images show almost the same signal. f T1-weighted image shows no adipose high signal intensity. An asterisk denotes the referred lytic lesion. g DIXON-based fat image shows almost complete loss of fat high signal in the bone marrow and soft tissue