This is one of the initial signs of renal disease.Easy bruising and abnormal bleeding tendencies are also described in initial diagnosis. This supports the common misdiagnosis for chronic idiopathic thrombocytopaenia purpura in patients with Epstein syndrome. These symptoms are often noticed in early childhood due to the congenital cause of the disease. Treatment
Epstein syndrome is regarded as a refractory disease. Treatments include renal transplantation, however, this may become problematic as patients low platelet count (thrombocytopaenia) increase the risks of complications in surgery. Successful Renal transplants can arise from cadavers or from live donors. To minimize the risk of patients losing too much blood during the perioperative period, HLA-matched platelet infusions can be used to maintain satisfactory platelet levels. Nephritis involves the inflammation of the kidney and this inflammation results in a reduced ability to filter blood. In order to ensure the transplanted kidney is recognised as ‘self’ by Major Histocompatibility cells, immunosuppression drugs are used post operation. Immunosuppression medication may include calcineurin inhibitor, antimetabolite and methylprednisolone and assist in suppressing the immune systems response to the transplanted kidney

Maintenance
As a result of nephritis, a healthy blood pressure becomes difficult to maintain and hence medication including vasopressin may be prescribed to maintain blood pressure. Severe nephritis may mean kidney dialysis is required to ensure the blood is being filtered. This may include either peritoneal dialysis or haemodialysis, however, haemodialysis is most common as Epstein syndrome patients will often eventually have renal transplants. Peritoneal dialysis involves blood being filtered through a membrane in the abdomen (peritoneum).
An intraneural perineurioma is a rare benign tumor within the sheath of a single nerve that grows but typically does not recur or metastasize. These lesions are only composed of perineurial cells, cloned from a single cell. They are distinct from schwannoma and neurofibroma. Intraneural perineurioma is a neoplastic proliferation of perineurial cells with unique immunohistochemistry and ultrastructural features, and it is distinct from other onion bulb Schwann cell-derived entities. Despite harboring molecular abnormalities of the long arm of chromosome 22, intraneural perineurioma has not been associated with neurofibromatosis.Due to the involvement of one or more nerve fascicles, intraneural perineuriomas are distinguished by a localized, solitary expansion of peripheral nerves. These tumors develop slowly or stay stable over time. References
Further reading
Macarenco RS, Ellinger F, Oliveira AM (2007). "Perineurioma: a distinctive and underrecognized peripheral nerve sheath neoplasm". Archives of Pathology & Laboratory Medicine. 131 (4): 625–36. doi:10.1043/1543-2165(2007)131[625:PADAUP]2.0.CO;2. PMID 17425397. Kum YS, Kim JK, Cho CH, Kim HK (2009). "Intraneural reticular perineurioma of the hypoglossal nerve". Head Neck. 31 (6): 833–7. doi:10.1002/hed.20965. PMID 18972430. Chung JH, Jeong SH, Dhong ES, Han SK (2014). "Surgical removal of intraneural perineurioma arising in the brachial plexus using an interfascicular dissection technique". Arch Plast Surg. 41 (3): 296–9. doi:10.5999/aps.2014.41.3.296. PMC 4037780. PMID 24883285. Emory TS, Scheithauer BW, Hirose T, Wood M, Onofrio BM, Jenkins RB (1995). "Intraneural perineurioma. A clonal neoplasm associated with abnormalities of chromosome 22". Am J Clin Pathol. 103 (6): 696–704. doi:10.1093/ajcp/103.6.696. PMID 7785653.