Abstract
Sneddon’s syndrome (SNS) which originally was a clinical diagnosis, is now regarded as a common clinical manifestation of different disease entities. It has been divided into idiopathic, autoimmune and thromboembolic subsets or in systemic lupus erythematosus (SLE)-associated, antiphospholipid syndrome (APS)-associated and primary forms. Familial occurrence of Sneddon’s syndrome is rare. We present a familial case of Sneddon’s syndrome with inflammatory disease pattern, early disease onset and association with autoimmune thyroid disease and anticardiolipin antibodies. Although most authors reporting on adult cases of SNS consider it a non-inflammatory, thromboembolic process, the study of cases with early onset brings attention to the possible inflammatory origin of the syndrome.
Similar content being viewed by others
Abbreviations
- SNS:
-
Sneddon’s syndrome
- APS:
-
Antiphospholipid syndrome
- aPL:
-
Antiphospholipid antibodies
- aCL:
-
Anticardiolipin antibodies
- SLE:
-
Systemic lupus erythematosus
References
Sneddon IB (1965) Cerebrovascular lesions and livedo reticularis. Br J Dermatol 77:180–185
Wohlrab J, Fischer M, Marsch WCh (2001) Aktuelle Diagnostik des Sneddon-Syndroms. Dtsch Med Wochenschr 126:725–728
Zelger B, Sepp N, Stockhammer G et al (1993) Sneddon’s syndrome. A long term follow-up of 21 patients. Arch Dermatol 129:437–447
Frances C, Papo T, Wechsler B, Laporte J-L, Biousse V, Piette J-C (1999) Sneddon syndrome with or without antiphospholipid antibodies. A comparative study in 46 patients. Medicine (Baltimore) 78:209–219
Gottloeber P, Bezold G, Schaer A et al (2000) Sneddon’s syndrome in a child. Br J Dermatol 142:374–375
Zipper S, Lambert S, Seemann W-R, Baer U, Schlisske K (2000) Sneddon-Syndrom: Vaskulitis oder thrombotische Vaskulopathie? Med Klin 95:158–162
Boortz-Marx RL, Clark HB, Taylor S, Wesa KM, Anderson DC (1995) Sneddon’s syndrome with granulomatous leptomeningeal infiltration. Stroke 26:492–495
Grattan CEH, Burton JL, Boon AP (1989) Sneddon syndrome (livedo reticularis and cerebral thrombosis) with livedo vasculitis and anticardiolipin antibodies. Br J Dermatol 120:441–447
Sepp N, Zelger B, Schuler G, Romani N, Fritsch P (1995) Sneddon’s syndrome—an inflammatory disorder of small arteries followed by smooth muscle proliferation. Immunohistochemical and ultrastructural evidence. Am J Surg Pathol 19:448–453
Asherson RA, Khamashta MA, Gil A et al (1989) Cerebrovascular disease and antiphospholipid antibodies in systemic lupus erythematosus, lupus-like disease and the primary antiphospholipid syndrome. Am J Med 86:391–399
Schellong SM, Weissenborn K, Niedermayer J et al (1997) Classification of Sneddon’s syndrome. Vasa 26:215–221
Rehany U, Kassif Y, Rumelt S (1998) Sneddon’s syndrome: neuro-ophthalmologic manifestations in a possible autosomal recessive pattern. Neurology 51:1185–1187
Pettee AD, Wasserman BA, Adams NL et al (1994) Familial Sneddon’s syndrome: clinical, hematologic and radiographic findings in two brothers. Neurology 44:399–405
Murese N, Kanda M, Satoi H, Kaji R, Akiguchi I (1996) Familial Biswanger-type encephalopathy with Sneddon syndrome. Rinsho Shinkeigaku 36:336–340
Rebollo M, Val JF, Garijo F, Quintana F, Bereciano J (1983) Livedo reticularis and cerebrovascular lesions (Sneddon’s syndrome). Clinical, radiological and pathological features in eight cases. Brain 106:965–979
Goel N, Ortel TL, Bali D et al (1999) Familial antiphospholipid antibody syndrome. Criteria for disease and evidence for autosomal dominant inheritance. Arthritis Rheum 42:318–327
Hademonos GJ, Alberts MJ, Awad I et al (2001) Advances in the genetics of cerebrovascular disease and stroke. Neurology 56:997–1008
Stockhammer G, Felber R, Zelger B et al (1993) Sneddon’s syndrome: diagnosis by skin biopsy and MRI in 17 patients. Stroke 24:685–690
Asherson RA, Cervera R (2001) In: Asherson RA, Cervera R (eds) Vascular manifestations of systemic autoimmune diseases, 1st edn. CRC Press, Boca Raton, pp 300–301
Zaccariotti VA, Martins LF, da Costa V et al (1995) Sneddon’s syndrome: report of 3 cases. Arq Neuropsiquiatr 53:82–87
Zelger B, Sepp N, Schmid KW, Hintner H, Klein G, Fritsch PO (1992) Life history of cutaneous vascular lesions in Sneddon’s syndrome. Hum Pathol 23:668–675
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Szmyrka-Kaczmarek, M., Daikeler, T., Benz, D. et al. Familial inflammatory Sneddon’s syndrome—case report and review of the literature. Clin Rheumatol 24, 79–82 (2005). https://doi.org/10.1007/s10067-004-0981-9
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10067-004-0981-9