Mucolipidosis II: correlation between radiological features and histopathology of the bones.

Twelve cases of Mucolipidosis II (I-cell disease) with a wide range of severity of skeletal involvement were studied. Pathological findings in two cases provided helpful information in understanding the radiographic features of dysostosis multiplex. Inhibition of the growth plate cartilage calcification and rickets-like lesions were observed in the metaphyses. Enhanced subperiosteal remodelling and paratrabecular fibrosis were also evident in the diaphyses. High levels of parathormone were found in one case. This finding supports the hypothesis that bone lesions may be secondary, at least in part, to damage in such viscera as the kidney and/or the liver and that they are mediated by vitamin D and parathormone

Langerhans cell histiocytosis of the sternum

We report a rare case of Langerhans cell histiocytosis involving the sternum. The patient was a 12-year-old girl presenting with anterior chest pain and swelling. Radiographs and computed tomography showed an osteolytic lesion in the sternum. Technetium bone scintigraphy revealed increased uptakes in the sternum, the greater trochanter of the right femur, and the right distal tibia. Incisional biopsy for the sternum lesion was performed, and the histopathologic diagnosis was Langerhans cell histiocytosis. She was treated with chemotherapy and the symptoms disappeared