Skip to main content
Top
Published in: Rheumatology International 5/2021

01-05-2021 | Antiphospholipid Syndrome | Cases with a Message

Sneddon syndrome: under diagnosed disease, complex clinical manifestations and challenging diagnosis. A case-based review

Authors: Steve S. Kong, Azin Azarfar, Neha Bhanusali

Published in: Rheumatology International | Issue 5/2021

Login to get access

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.
Literature
7.
go back to reference 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):215–221PubMed 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):215–221PubMed
9.
go back to reference 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):654–660PubMed 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):654–660PubMed
11.
go back to reference Uthman IW, Khamashta MA (2006) Livedo racemosa: a striking dermatological sign for the antiphospholipid syndrome. J Rheumatol 33(12):2379–2382PubMed Uthman IW, Khamashta MA (2006) Livedo racemosa: a striking dermatological sign for the antiphospholipid syndrome. J Rheumatol 33(12):2379–2382PubMed
17.
go back to reference Bolognia JL, Schaffer JV, Cerroni L (2018) Neoplasms of the skin. Dermatology, 4th edn. Elsevier, New York, pp 1989–2020 Bolognia JL, Schaffer JV, Cerroni L (2018) Neoplasms of the skin. Dermatology, 4th edn. Elsevier, New York, pp 1989–2020
21.
go back to reference 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):633–639CrossRef 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):633–639CrossRef
25.
go back to reference Lao M, Setty S, Foss C (2001) Antiphospholipid antibody syndrome. A literature review. Minn Med 84(4):42–46PubMed Lao M, Setty S, Foss C (2001) Antiphospholipid antibody syndrome. A literature review. Minn Med 84(4):42–46PubMed
26.
go back to reference 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.2016.03.011CrossRefPubMed 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.​011CrossRefPubMed
27.
go back to reference Riva N, Gatt A (2019) Update on the diagnosis and anticoagulant treatment of the antiphospholipid syndrome. EMJ Rheumatol 6(1):101–111 Riva N, Gatt A (2019) Update on the diagnosis and anticoagulant treatment of the antiphospholipid syndrome. EMJ Rheumatol 6(1):101–111
Metadata
Title
Sneddon syndrome: under diagnosed disease, complex clinical manifestations and challenging diagnosis. A case-based review
Authors
Steve S. Kong
Azin Azarfar
Neha Bhanusali
Publication date
01-05-2021
Publisher
Springer Berlin Heidelberg
Published in
Rheumatology International / Issue 5/2021
Print ISSN: 0172-8172
Electronic ISSN: 1437-160X
DOI
https://doi.org/10.1007/s00296-020-04625-1

Other articles of this Issue 5/2021

Rheumatology International 5/2021 Go to the issue