This is the first case report of abnormal pigmentation (darkening) of the human peripheral nerve. Gross anatomically, only the posterior cutaneous nerve of thigh showed abnormality bilaterally. Light microscopical examinations showed that the pigmentation was localized in the perineurial epithelial sheath and endoneurial vessels. The pigmented loci were hematoxylinophilic, PAS positive, Schmorl positive, Masson-Fontana negative, Puts negative, diazo negative, and showed neither autofluorescence nor birefringence, denying the presence of melanin, hemosiderin, lipofuscin, and any other well known pigments. All of the pigmented loci and many other non-pigmented areas in histological sections were von Kossa positive indicating the presence of calcium. Electron microscopy and microanalysis showed that calcium apatite crystallites occurred in and adjacent to the perineural epithelium, the endothelium of endoneurial vessels, the axoplasm and Schwann cells. The crystallites appeared originating intracellularly, at places penetrating the cytolemma and projecting toward the interstice of the nerve. Whether a portion of nerve fasciculus appears pigmented or not was directly correlated with the presence or absence of extracellularly projecting calcium crystallites. It was assumed that an unknown kind of pigment accumulated on the extracellularly projecting calcium deposits, but not on those deposits being confined intracellularly.
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