Summary
Progressive systemic sclerosis (PSS) is a chronic inflammatory disease of the connective tissue with involvement of the skin and other organs. The disease is characterized by an abnormal accumulation of collagen in all tissues and by microangiopathy. The involvement of the peripheral nervous system during PSS is very unusual and few cases are reported in the literature. A morphological study on the neuropathy associated with sclerodermia has been performed in rare cases. In this paper we demonstrate the role that the vascular lesions have in the pathogenesis of neuropathy during scleroderma. In particular, the primary role of the peripheral microangiopathy during PSS (observed in different clinical cases) is verified.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Ashworth B, Tait GBW (1971) Trigeminal neuropathy in connective tissue disease. Neurology 21:609–641
Beighton P, Gumpel JM, Cornes NGM (1968) Prodomal trigeminal sensory neuropathy in progressive systemic sclerosis. Ann Rheum Dis 27:367–369
Camilleri JP, Fiesvinger JN, Debure C, Bruneral P, Tricottet V, Kazandjian S, Housset H (1984) Fine structural capillary changes and basal lamina. Thickening in scleroderma (progressive systemic sclerosis and Raynaud's disease). Pathol Res Pract 178:230–236
Comi G, Comola M, Nemmi R, Canal N (1982) Study of a case of neuropathy associated with progressive systemic sclerosis. Acta Neurol (Napoli) 4:258
Currie DM, Bradshaw DC (1979) Polyneuropathy in mixed connective tissue disease presenting as progressive systemic sclerosis (scleroderma): case report and literature review. Arch Phys Med Rehabil 60:549
Dimitriu R, Stamatoiu I, Nicolae I, Ciobanu I (1977) Scleroderma and its relationship to nervous system disease. Report of a case. Rom Med Neurol Psychiatry 15:117–123
Di Trapani G, Pocchiari M, Masullo C, Albanese A, Tulli A (1982) Peripheral neuropathy in the course of progressive systemic sclerosis: light and ultrastructural study. Ital J Neurol Sci 4:341–348
Farrel AD, Medseger AT (1982) Trigeminal neuropathy in progressive system sclerosis. Am J Med 73:57–62
Gordon RM, Silverstein A (1970) Neurologic manifestations in progressive systemic sclerosis. Arch Neurol 22:126–134
Kibler RF, Rose FG (1960) Peripheral neuropathy in the collagen disease. A case of scleroderma neuropathy. Br Med J 1:1781–1784
Leinwand I, Duryee AW, Richter MN (1954) Scleroderma (based on a study of over 150 cases). Ann Intern Med 41:1003–1041
O'Leary PA, Nomland R (1930) A clinical study of 103 cases of scleroderma. Am J Med Sci 180:95
Richter RL (1954) Peripheral neuropathy and connective tissue disease. J Neuropathol Exp Neurol 13:168–180
Teasdall RD, Frayha RA, Shulman LE (1980) Cranial nerve involvement in systemic sclerosis (scleroderma). A report of 10 cases. Medicine (Baltimore) 59:149–159
Tuffanelli DL, Winkelmann RK (1961) Systemic scleroderma. A clinical study of 727 cases. Arch Dermatol 84:49–61
Zulch KJ (1959) Über die Skleroneuropathie, die Mitbeteiligung der peripheren Nerven bei der allgemeinen progressiven Sklerodermie. Mit Versuch einer pathogenetischen Deutung. Dtsch Z Nervenklinik 179:1–21
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Di Trapani, G., Tulli, A., La Cara, A. et al. Peripheral neuropathy in course of progressive systemic sclerosis. Acta Neuropathol 72, 103–110 (1986). https://doi.org/10.1007/BF00685970
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00685970