Abstract
Herein, we report a case-based review of the Sneddon Syndrome (SS), a rare chronic condition which affects small to medium blood vessels. It is known by its skin presentation, livedo racemosa (LRC), and the relapsing cerebrovascular events. However, neither LRC nor cerebrovascular events are exclusive to SS. A 36-year-old female with history of mitral valve prolapse, hypothyroidism, Raynaud phenomenon, hypertension, migraines, and four episodes of transient ischemic attacks (TIA), presented to our clinic with new skin findings, suggestive of LRC. Based on her previous history, current presentation and skin biopsy results, she was diagnosed with SS secondary to antiphospholipid syndrome. The present report illustrates the difficulty in recognizing SS and how the heterogeneity of the disease may be contributing to the difficulty making a distinct diagnosis.
Similar content being viewed by others
References
Wu S, Xu Z, Liang H (2014) Sneddon's Syndrome: a comprehensive review of the literature. Orphanet J Rare Dis 9:215. https://doi.org/10.1186/s13023-014-0215-4
Fabiani G, Martins RF, Koppe GL, Demartini Z Jr, Gatto LAM (2018) Cognitive and psychiatric changes as first clinical presentation in Sneddon Syndrome. Dement Neuropsychol 12(2):216–219. https://doi.org/10.1590/1980-57642018dn12-020016
Bolayir E, Yilmaz A, Kugu N, Erdogan H, Akyol M, Akyuz A (2004) Sneddon's Syndrome: clinical and laboratory analysis of 10 cases. Acta Med Okayama 58(2):59–65. https://doi.org/10.18926/amo/32100
Tietjen GE, Al-Qasmi MM, Shukairy MS (2002) Livedo reticularis and migraine: a marker for stroke risk? Headache 42(5):352–355. https://doi.org/10.1046/j.1526-4610.2002.02106.x
Frances C, Papo T, Wechsler B, Laporte JL, Biousse V, Piette JC (1999) Sneddon syndrome with or without antiphospholipid antibodies. A comparative study in 46 patients. Medicine 78(4):209–219. https://doi.org/10.1097/00005792-199907000-00001
Sneddon IB (1965) Cerebro-vascular lesions and livedo reticularis. Br J Dermatol 77:180–185. https://doi.org/10.1111/j.1365-2133.1965.tb14628.x
Schellong SM, Weissenborn K, Niedermeyer J, Wollenhaupt J, Sosada M, Ehrenheim C, Lubach D (1997) Classification of Sneddon's syndrome. VASA Zeitschrift fur Gefasskrankheiten 26(3):215–221
Forchhammer S, Metzler G, Ghoreschi K (2018) Long-term follow-up of early-onset Sneddon syndrome: a case report. JAAD Case Rep 4(9):880–882. https://doi.org/10.1016/j.jdcr.2018.08.008
Orac A, Artenie A, Toader MP, Harnagea R, Dinu-Mitrofan D, Grigorovici M, Ungureanu G (2014) Sneddon syndrome: rare disease or under diagnosed clinical entity? Review of the literature related to a clinical case. Rev Med Chir Soc Med Nat Iasi 118(3):654–660
Kraemer M, Linden D, Berlit P (2005) The spectrum of differential diagnosis in neurological patients with livedo reticularis and livedo racemosa. A literature review. J Neurol 252(10):1155–1166. https://doi.org/10.1007/s00415-005-0967-9
Uthman IW, Khamashta MA (2006) Livedo racemosa: a striking dermatological sign for the antiphospholipid syndrome. J Rheumatol 33(12):2379–2382
Tietjen GE, Al-Qasmi MM, Gunda P, Herial NA (2006) Sneddon's syndrome: another migraine-stroke association? Cephalalgia 26(3):225–232. https://doi.org/10.1111/j.1468-2982.2005.01032.x
Frances C, Piette JC (2000) The mystery of Sneddon syndrome: relationship with antiphospholipid syndrome and systemic lupus erythematosus. J Autoimmun 15(2):139–143. https://doi.org/10.1006/jaut.2000.0418
Bottin L, Frances C, de Zuttere D, Boelle PY, Muresan IP, Alamowitch S (2015) Strokes in Sneddon syndrome without antiphospholipid antibodies. Ann Neurol 77(5):817–829. https://doi.org/10.1002/ana.24382
Zelger B, Sepp N, Stockhammer G, Dosch E, Hilty E, Öfner D, Aichner F, Fritsch PO (1993) Sneddon's Syndrome: a long-term follow-up of 21 patients. Arch Dermatol 129(4):437–447. https://doi.org/10.1001/archderm.1993.01680250049006
Aquino Gondim Fde A, Leacock RO, Subrammanian TA, Cruz-Flores S (2003) Intracerebral hemorrhage associated with Sneddon's syndrome: is ischemia-related angiogenesis the cause? Case report and review of the literature. Neuroradiology 45(6):368–372. https://doi.org/10.1007/s00234-003-0990-4
Bolognia JL, Schaffer JV, Cerroni L (2018) Neoplasms of the skin. Dermatology, 4th edn. Elsevier, New York, pp 1989–2020
Al Meshari K, Al Eisa A, Akhtar M (1995) Sneddon's Syndrome: a systemic arterio-occlusive disorder. Am J Kidney Dis 26(2):368–372. https://doi.org/10.1016/0272-6386(95)90659-2
Stone J, Bhattacharya J, Walls TJ (2001) Divry-Van Bogaert syndrome in a female: relationship to Sneddon's Syndrome and radiographic appearances. Neuroradiology 43(7):562–564. https://doi.org/10.1007/s002340000534
Sajjan VV, Lunge S, Swamy MB, Pandit AM (2015) Livedo reticularis: a review of the literature. Indian Dermatol Online J 6(5):315–321. https://doi.org/10.4103/2229-5178.164493
Lubach D, Schwabe C, Weissenborn K, Hartung K, Creutzig A, Drenk F (1990) Livedo racemosa generalisata: an evaluation of thirty-four cases. J Am Acad Dermatol 22(4):633–639
Gibbs MB, English JC 3rd, Zirwas MJ (2005) Livedo reticularis: an update. J Am Acad Dermatol 52(6):1009–1019. https://doi.org/10.1016/j.jaad.2004.11.051
Levine JS, Branch DW, Rauch J (2002) The antiphospholipid syndrome. N Engl J Med 346(10):752–763. https://doi.org/10.1056/NEJMra002974
Whitaker KL (2017) Antiphospholipid antibody syndrome: the difficulties of diagnosis. Jaapa 30(12):10–14. https://doi.org/10.1097/01.Jaa.0000526771.67820.59
Lao M, Setty S, Foss C (2001) Antiphospholipid antibody syndrome. A literature review. Minn Med 84(4):42–46
Bersano A, Morbin M, Ciceri E, Bedini G, Berlit P, Herold M, Saccucci S, Fugnanesi V, Nordmeyer H, Farago G, Savoiardo M, Taroni F, Carriero M, Boncoraglio Giorgio B, Perucca L, Caputi L, Parati Eugenio A, Kraemer M (2016) The diagnostic challenge of Divry van Bogaert and Sneddon Syndrome: report of three cases and literature review. J Neurol Sci 364:77–83. https://doi.org/10.1016/j.jns.2016.03.011
Riva N, Gatt A (2019) Update on the diagnosis and anticoagulant treatment of the antiphospholipid syndrome. EMJ Rheumatol 6(1):101–111
Flöel A, Imai T, Lohmann H, Bethke F, Sunderkötter C, Droste DW (2002) Therapy of Sneddon Syndrome. Eur Neurol 48(3):126–132. https://doi.org/10.1159/000065510
Kraemer M, Baumgaertel MW, Berlit P (2007) Miscarriage, peripheral thromboses and aortic aneurysm in antiphospholipid-antibody-negative Sneddon's Syndrome. J Neurol 254(11):1599–1600. https://doi.org/10.1007/s00415-007-0568-x
Wohlrab J, Fischer M, Wolter M, Marsch WC (2001) Diagnostic impact and sensitivity of skin biopsies in Sneddon's Syndrome. A report of 15 cases. Br J Dermatol 145(2):285–288. https://doi.org/10.1046/j.1365-2133.2001.04348.x
Funding
This case-based review did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. This research was supported (in whole or in part) by HCA Healthcare and/or an HCA Healthcare affiliated entity. The views expressed in this publication represent those of the author(s) and do not necessarily represent the official views of HCA Healthcare or any of its affiliated entities.
Author information
Authors and Affiliations
Contributions
All authors contributed to the study conception, design, and manuscript writing. SSK: data collection and review of patients’ data, writing of manuscript including editing and revisions, and literature review at all stages of its production. AA: review of patients’ data, writing of manuscript including editing and revisions, and literature review at all stages of its production. NB: data collection and review of patients’ data, writing of manuscript including editing and revisions, and literature review at all stages of its production. All authors take full responsibility for all aspects of the study and the final manuscript.
Corresponding author
Ethics declarations
Conflict of interest
The authors declare that there is no conflict of interest.
Informed consent
The patient has provided permission/consent to publish her case, and the identity of the patient has been protected.
Research involving human participants and/or animals
This is a case report and a literature review and it did not involve any human/and or animal participants.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Kong, S.S., Azarfar, A. & Bhanusali, N. Sneddon syndrome: under diagnosed disease, complex clinical manifestations and challenging diagnosis. A case-based review. Rheumatol Int 41, 987–991 (2021). https://doi.org/10.1007/s00296-020-04625-1
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00296-020-04625-1