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The cause of the disease is unknown. It has been suggested that SHML may represent an abnormal reactive response of histiocytes to an infectious agent. EBV and HHV-6 have been considered as possible causative infection agents since elevated titers of HHV-6 and EBV antibodies have been detected in blood, and HHV-6 has been demonstrated in the involved lymph nodes. PCR studies from the lesional skin have been negative. Clonality studies have given evidence of a polyclonal nature of the histiocytes in SHML (8-10).

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