Summary
We present a case of Sneddon's syndrome with high titers of antiphospholipid antibodies (APLA), in which the leading symptom was an incapacitating memory defect. MRI revealed vasculitic lesions of the central nervous system (CNS). Therefore immunosuppressive therapy was started with steroids and cyclophosphamide pulses. The transient beneficial effects of such a therapy will be discussed.
Similar content being viewed by others
References
Sneddon, I.B. Cerebrovascular lesions and livedo reticularis. Br J Dermatol 1965, 77, 180–185.
Lubach, D., Schwabe, C., Weissenborn, K., Hartung, K., Creutzig, A., Drenk, F. Livedo racemosa generalisata: an evaluation of thirty-four cases. J Am Acad Dermatol 1990, 22, 633–639.
Englert, H.J., Loizou, S., Derue, G.G., Walport, J.M. Clinical and immunologic features of livedo reticularis in lupus: a case control study. Am J Med 1989, 87, 408–410.
Kalashnikova, L.A., Nasonov, E.L., Kushekbaeva, A.E., Gracheva, L.A. Anticardiolipin antibodies in Sneddon's syndrome. Neurology 1990, 40, 464–467.
Levine, S.R., Langer, S.L., Albers, J.W., Welch, K.M.A. Sneddon's syndrome: an antiphospholipid antibody syndrome? Neurology 1988, 38, 798–800.
Montalban, J., Ordi, J., Barquinero, J., Villardel, M. Sneddon's syndrome and anticardiolipin antibodies. Stroke 1988, 19, 785–786.
Vargas, J.A., Yebra, M., Pascual, M.L. Antiphospholipid antibodies and Sneddon's syndrome. Am J Med 1989, 87, 597.
Schulze-Lohoff, F., Krapf, F., Bumester, G.R., Kölbe, K., Nüßlein, H.G., Rödl, W. IgM-containing immune complexes and antiphospholipid antibodies in patients with Sneddon's syndrome. Rheumatol Int 1989, 9, 43–48.
Alegre, V.A., Winkel, R.K., Gastineau, D.A. Cutaneous thrombosis cerebrovascular thrombosis, and lupus anticoagulant — the Sneddon syndrome. Report of 10 cases. Int J Dermatol 1990, 29, 45–49.
Asherson, R.A., Kamashta, M.A. Sneddon's syndrome and primary antiphospholipid syndrome (PAPS)(letter). Br J Dermatol 1990, 122, 115–116.
Otoyama, K., Katayama, I., Suzuki, Y., Tone, T., Nishioka, K., Nishiyama, S. A case of Sneddon's syndrome with positive ANA and anti-cardiolipin antibodies: primary antiphospholipid syndrome? J Dermatol 1990, 17, 489–492.
Englert, H., Derue, G., Hughes, G.R. Neurology and the lupus anticoagulant. Eur Neurol 1985, 24, 422–425.
Harris, E.N., Gharavi, A.E., Mackworth-Young, C.G., Patel, B.M., Derue, G., Hughes, G.R. Lupoid sclerosis: a possible pathogenetic role for antiphospholipid antibodies. Ann Rheum Dis 1985, 44, 281–283.
Harris, E.N., Gharavi, A.E., Hughes, G.R. Antiphospholipid antibodies. Clin Rheum Dis 1985, 11, 591–609.
Levine, S.R., Welch, K.M. The spectrum of neurologic disease associated with antiphospholipid antibodies. Arch Neurol 1987, 44, 876–883.
Hughes, G.R., Harris, E.N., Gharavi, A.E. The anticardiolipin syndrome. J Rheumatol 1986, 13, 486–489.
Love, P.E., Santoro, S.A. Antiphospholipid antibodies: anticardiolipin and the lupus anticoagulant in systemic lupus erythematosus (SLE) and in non-SLE disorders. Ann Intern Med 1990, 112, 682–698.
Hart, R.G., Miller, V.T., Coull, B.M., Bril, V. Cerebral infarction associated with lupus anticoagulant-preliminary report. Stroke 1984, 15, 114–118.
Asherson, R.A., Khamashta, A., Gil, A., Vasquez, J.J., Chan, O., Baguley, E., Hughes, G.R.V. Cerebrovascular disease and antiphospholipid antibodies in systemic lupus erythematosus, lupus-like disease and the primary antiphospholipid syndrome. Am J Med 1989, 86, 391–399.
Lockshin, M. Antiphospholipid antibody and antiphospholipid antibody syndrome. Curr Opinion Rheumatol 1991, 3, 797–802.
Bajaj, S.P., Rapaport, S.L., Frierer, D.S., Herbst, K.D., Schwartz, D.B. A mechanism for the hypothrombinemia of the acquired hypoprothrombinemia-lupus anticoagulant syndrome. Blood 1983, 61, 684–692.
Malia, R.G., Kitchen, S., Greaves, M., Preston, F.E. Inhibition of activated protein C and its cofactor protein S by antiphospholipid antibodies. Br J Haematol 1990, 76, 101–107.
Carreras, L.O., Vermylen, J.G. Lupus anticoagulant and thrombosis: possible role of inhibition of prostacyclin formation. Thromb Haemost 1982, 48, 28–40.
San Felippo, M.J., Drayna, C.J. Prekallikrein inhibition associated with the lupus anticoagulant. Am J Clin Pathol 1982, 77, 275–279.
Harris, E.N., Gharavi, A.E., Hedge, U., Derue, G., Morgan, S.M., Englert, H., Chon, J.K.H., Asherson, R.A., Hughes, G.R.V. Anticardiolipin antibodies in autoimmune thrombocytopenic purpura. Br J Haematol 1985, 59, 231–234.
Khamashta, M.A., Harris, E.N., Gharavi, A.E., Derue, G., Gil, A., Vasquez, J.J., Hughes, G.R. Immune-mediated mechanism for thrombosis: antibody binding to platelet membranes. Ann Rheum Dis 1988, 47, 849–854.
Bruyn, R.P.M., Van der Veen, J.P.N., Danke, A.J.M., Valk, K., Wollers, E.C. Sneddon's syndrome. Case report and literature review. J Neurol Sci 1987, 79, 243–253.
Rumpl, E., Neuhofer, J., Pallua, A., Willeit, J., Vogl, G., Stamfel, G., Platz, T. Cerebrovascular lesions and livedo reticularis (Sneddon's syndrome) a progressive cerebrovascular disorder? J Neurol 1985, 231, 324–330.
Rump, J.A., Lang, B., Engler, H., Peter, H.H. Primary antiphospholipid syndrome (PAPS) Rheumatol Int 1991, 10, 255–260.
Parke, A., Maier, D.M., Wilson, D., Andreoli, J., Ballow, M. Intravenous gammaglobulin, antiphospholipid antibodies and pregnancy. Ann Intern Med 1989, 110, 495–496.
Asherson, R.A., Chan, J.K.H., Harris, E.N. Anticardiolipin antibody, recurrent thromboses and warfarin withdrawal. Ann Rheum Dis 1985, 44, 823–825.
Landi, G., Calloni, M.V., Sabbadini, M.G., Munnucci, P.M., Candelise, L. Recurrent ischemic attacks in two young adults with lupus anticoagulant. Stroke 1983, 14, 377–379.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Mesa, H.A., Lang, B., Schumacher, M. et al. Sneddon's syndrome and phospholipid antibodies. Clin Rheumatol 12, 253–256 (1993). https://doi.org/10.1007/BF02231537
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF02231537