Multiple Pacinian neurofibromas involving the three phalanges of the ring finger of the left hand in a 60-year-old woman are described. The abnormal proliferation of Vater-Pacini corpuscles in various stages of differentiation was associated with marked changes of the glomus type of arterio-venous anastomoses of the finger-tip. The close relationship between the new proliferated Vater-Pacini corpuscles, their altered baresthetic function and the pathological shunting of blood account for the painful Raynaud-like symptoms. The name 'Pacinian neurofibromatosis' is proposed.
Hoyer-Grosser's organs were studied in human digital biopsies. The fine structure of both the supplying arteries and collecting veins was found to be inconspicuous. Endothelial cells in the AV canals form a continuous layer. They are characterized by their rich content of specific organelles (Weibel-Palade bodies), especially in the venous segments. The epitheloid zone is composed of a variety of ramified smooth muscle cells (RSM). These appear either dense, when well provided with bundles of myofilaments, or clear, when including only a few myofilaments. The nuclei of dense RSM show condensed chromatin, while those of clear RSM are larger with loose chromatin texture. In addition, all transitional forms occur. Cell organelles are rarely seen within all types of RSM. The cytoplasmic processes reach other as well as endothelial cells. The preservation of our material did not allow observation of specialized membrane contacts in these zones. All RSM are invested with a regular basal lamina and well provided with surface vesicles. Profiles of free basal lamina material and faint collagen (argyrophil) fibers are seen in the wide intercellular spaces. RSM poor in myofilaments are interpreted to represent "epitheloid cells" of light microscopy. Their number constantly decreases from the arterial segment of the AV canal to the venous segment. Here the cytoplasmic processes of RSM become less pronounced and the wall of the anastomotic segment continuously changes to that of the collecting vein. Dense RSM rich in myofilaments are compared with pacemaker cells found inthe ureter. Both internal and external elastic membranes are absent in AV canals. A dense network of unmyelinated nerve fibers is found in the adventitial layer of the AV canal, especially in its arterial segment. The axons branch out from small dermal nerves which also contain two or three myelinated axons. The myelin sheaths terminate where the axons reach the adventitia of the AV canals. Axon varicosities filled with mitochondria are thought to be terminals of myelinated axons and are interpreted as receptory. Axon varicosities with synaptic-type vesicles are assumed to be terminals of sympathetic and para sympathetic fibers. All axon profiles are confined to the adventitial layer of the anastomotic segment.
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