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Presented by Edward McCarthy, M.D. and prepared by Marc Halushka M.D., Ph.D.
Case 6: A 45-year old man had pain in both his legs.
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1. Question
Week 163: Case 6
A 45-year old man had pain in both his legs. Radiographs demonstrated defuse radiodensity in a zonal pattern that involved both femurs. In addition, this gentleman had defused radiodensities involving many other long bones. A biopsy showed numerous histiocytic-like cells, and an S100 stain was negative.images/Halushka/conf121503/case6image1.jpg
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images/Halushka/conf121503/case6image4.jpgCorrect
Answer: Erdheim-Chester disease
Histology: Erdheim-Chester disease is a systemic proliferation of histiocytes that involve many bones of the skeleton. In any one bone, a radiographic pattern is similar to chronic osteomyelitis. However, unlike chronic osteomyelitis, many bones in the skeleton are involved. In addition, wound cultures in Erdheim-Chester disease are negative.
Discussion: The lesion is not osteomyelitis because of the multiple bones involved and because cultures were negative. Otherwise, the histologic pattern of any one lesion (as well as the radiographic pattern) could be highly suggestive of osteomyelitis.
The lesion is not Langerhans’ cell histiocytosis because the S100 stain is negative. Langerhans’ cell histiocytosis is a histiocytic proliferation that may involve multiple bones. However, the lesions are usually punched out and lytic, and they do not form a pattern of irregular radiodensity as in this case.
Rosai-Dorfman disease is also a histiocytic proliferation that occasionally may involve one or more bones. This disease usually involves lymph nodes, but has been reported in a wide variety of visceral locations, including bone. S100 stain is positive in Rosai-Dorfman disease, and this case could not be given that diagnosis.
Incorrect
Answer: Erdheim-Chester disease
Histology: Erdheim-Chester disease is a systemic proliferation of histiocytes that involve many bones of the skeleton. In any one bone, a radiographic pattern is similar to chronic osteomyelitis. However, unlike chronic osteomyelitis, many bones in the skeleton are involved. In addition, wound cultures in Erdheim-Chester disease are negative.
Discussion: The lesion is not osteomyelitis because of the multiple bones involved and because cultures were negative. Otherwise, the histologic pattern of any one lesion (as well as the radiographic pattern) could be highly suggestive of osteomyelitis.
The lesion is not Langerhans’ cell histiocytosis because the S100 stain is negative. Langerhans’ cell histiocytosis is a histiocytic proliferation that may involve multiple bones. However, the lesions are usually punched out and lytic, and they do not form a pattern of irregular radiodensity as in this case.
Rosai-Dorfman disease is also a histiocytic proliferation that occasionally may involve one or more bones. This disease usually involves lymph nodes, but has been reported in a wide variety of visceral locations, including bone. S100 stain is positive in Rosai-Dorfman disease, and this case could not be given that diagnosis.
