Buschke-Ollendorff syndrome accidentally diagnosed after a left ankle sprain.

A 9-year-old girl was referred to our clinic for evaluation of a spherical sclerotic bone lesion in the distal metaphysis of the left tibia (Figure 1A). The lesion was accidentally found after a routine radiograph for a minor left ankle injury she sustained a week earlier. Physical examination revealed multiple, asymptomatic, symmetrically located skin lesions on either side of the lower back and proximal thighs. These lesions had smooth surface with disseminated texture and did not coalesce to form plaques (Figure 1B). No pain or pruritus was present. These findings have been present for the past 4 years but no diagnosis was made or any treatment initiated, since all the clinical and laboratory tests performed in the past were normal. Her past medical history was largely unremarkable as well as her family history for any type of skin disorders. Routine laboratory tests (blood cell counts, ESR, CRP, liver and renal function levels as well as calcium and phosphate levels) were within normal limits. A radiographic skeletal survey was ordered. Radiographs demonstrated multiple well defined solitary sclerotic lesions on the distal metaphysis of the left tibia and both radii, consistent with osteopoikilosis (Figure 1C). Further X-rays of hands, feet, lumbar spine and pelvis showed no abnormalities. Bone lesions exhibited no increased radioisotope uptake during the three phases of the bone scanning with Tc (Figure 2A). MRI of the left tibia revealed a solitary, well-circumscribed, lesion off 4 mm in diameter with no uptake of the paramagnetic substance. Periosteal reaction was not recognized in the examined area and the surrounding soft tissues were intact (Figure 2B). A punch biopsy of a skin lesion from her left thigh revealed thick collagen fibers through the dermis consistent with dermatofibrosis lenticularis disseminata. The characteristic bone J Musculoskelet Neuronal Interact 2014; 14(1):144-147