Cases reported "Erdheim-Chester Disease"

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1/2. erdheim-chester disease.

    A 38-year-old man presented with numerous dermal nodules, similar to xanthoma disseminatum, that were histologically consistent with his diagnosis of erdheim-chester disease, a non-Langerhans cell histiocytosis. Other cutaneous manifestations of this disease include eyelid xanthelasma, pretibial dermopathy and pigmented lesions of the lips and buccal mucosa. The histological diagnosis of erdheim-chester disease was originally made on the patient's retroperitoneal tissue, obtained at a laparotomy for surgical treatment of a presumed phaeochromocytoma, and confirmed by the pathognomonic long bone X-ray findings of this disease.
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keywords = tibia
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2/2. erdheim-chester disease of the breast associated with Langerhans-cell histiocytosis of the hard palate.

    We report a patient with Langerhans-cell histiocytosis (LCH) localized to the hard palate that was later proven to be associated with erdheim-chester disease (ECD), involving the right breast, skeleton, retroperitoneum and left orbit. The diagnosis was based on the symmetric osteosclerosis of the long bones diaphyses (tibias and fibulas), breast lump histopathological/immunohistochemical findings and retroperitoneum and left orbit images in magnetic resonance. Mammary involvement by ECD is an extremely rare condition, which should be differentiated from some benign and malignant mimickers, especially the histiocytoid type of breast carcinoma. Characteristic histological features plus clinical and radiographic information are needed to achieve a correct diagnosis. The ECD, its relation to the LCH and details of the breast lesion are discussed.
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