Subacute inflammatory polyneuropathy: two cases with plasmacytoid histiocytes in the endoneurium

Inflammatory polyneuropathies are mainly known by their acute form, Guillain-Barré syndrome, but there are also chronic cases and all are considered as having an autoimmune mechanism. In each form, peripheral nerve biopsies show scattered macrophages in the endoneurium and in certain cases macrophages invade the Schwann cell cytoplasm and destroy the myelin sheath. In rarer cases there is a primary axonal degeneration. The authors studied two chronic cases, which both exhibited mixed primitive axonal and demyelinating lesions, with peculiar histiocytes in the endoneurium. These rounded cells were characteristically well marked by KP1 but showed well-developed rough endoplasmic reticulum cysternae at ultrastructural examination. Such plasmacytoid histiocytes have mainly been studied in subacute lymphadenopathies and have been only briefly illustrated in a few cases of peripheral neuropathies due to Lyme disease or HIV infection. The two cases reported here had no associated diseases and probably correspond to a peculiar subacute autoimmune reaction.