Sneddon Syndrome

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Classification according to ICD-10
I77.8 Other specified diseases of arteries and arterioles
ICD-10 online (WHO version 2019)

The Sneddon syndrome is defined as a combination of a livedo racemosa generalisata with cerebrovascular events, especially strokes, especially in the case of cerebral microangiopathy. Sneddon syndrome is a disease that occurs predominantly in women ( gynecotropia ) ( female: male = 3: 1), which is characterized by a reticulate skin , temporary ( transient ) ischemic attacks (TIA) and headaches .

It is important to distinguish between the physiological livedo reticularis (e.g. in cold weather) and the pathological livedo racemosa. It is also important that Livedo racemosa can occur with a variety of internal and neurological diseases and not just with Sneddon syndrome. Therefore an intensive differential diagnostic clarification is necessary.

Histopathologically, there is a proliferation of the ( subintimal ) smooth muscle cells located under the vascular lining in medium-sized arteries , which leads to vascular occlusion.

In 20% of cases can be in the serum of patients autoantibodies called antiphospholipid antibodies prove.

There are no major studies on therapies; the therapeutic approaches are based on expert opinion.

literature

  • M. Kraemer, D. Linden, P. Berlit: The spectrum of differential diagnosis in neurological patients with livedo reticularis and livedo racemosa. A literature review . In: Journal of neurology . Volume 252, Number 10, October 2005, pp. 1155-1166, ISSN  0340-5354 . doi: 10.1007 / s00415-005-0967-9 . PMID 16133722 . (Review).
  • A. Zimpfer A u. a .: stroke and patches of skin . In: Schweiz Med Forum , 2004, 4, pp. 314-315; medicalforum.ch (PDF)
  • E. Lewandowska, T. Wierzba-Bobrowicz, T. Wagner, R. Bogusławska, A. Rudnicka, A. Leszczyńska, E. Pasennik, W. Lechowicz, T. Stepień, W. Kuran: Sneddon's syndrome as a disorder of small arteries with endothelial cells proliferation: ultrastructural and neuroimaging study. In: Folia neuropathologica / Association of Polish Neuropathologists and Medical Research Center, Polish Academy of Sciences . Volume 43, Number 4, 2005, pp. 345-354, ISSN  1641-4640 . PMID 16416398 .
  • D. Al Aboud, V. Broshtilova, K. Al Aboud, K. Al Hawsawi: Dermatological aspects of cerebrovascular diseases. In: Acta dermatovenerologica Alpina, Panonica, et Adriatica . Volume 14, Number 1, March 2005, pp. 9-14, ISSN  1318-4458 . PMID 15818440 . (Review).
  • M. Kume, H. Imai, M. Motegi, AB Miura, I. Namura: Sneddon's syndrome (livedo racemosa and cerebral infarction) presenting psychiatric disturbance and shortening of fingers and toes. In: Internal medicine (Tokyo, Japan) . Volume 35, Number 8, August 1996, pp. 668-673, ISSN  0918-2918 . PMID 8894746 . (Review).
  • SM Boesch, AL Plörer, AJ Auer, W. Poewe, FT Aichner, SR Felber, NT Sepp: The natural course of Sneddon syndrome: clinical and magnetic resonance imaging findings in a prospective six year observation study. In: Journal of Neurology, Neurosurgery, and Psychiatry . Volume 74, Number 4, April 2003, pp. 542-544, ISSN  0022-3050 . PMID 12640088 . PMC 173839 (free full text).
  • GE Tietjen, MM Al-Qasmi, P. Gunda, NA Herial: Sneddon's syndrome: another migraine-stroke association? In: Cephalalgia. Volume 26, Number 3, March 2006, pp. 225-232, ISSN  0333-1024 . doi: 10.1111 / j.1468-2982.2005.01032.x . PMID 16472327 . (Review).
  • SG Zipper, S. Lambert et al. a .: Sneddon syndrome: vasculitis or thrombotic vasculopathy? In: Medical Clinic. Volume 95, number 3, 2000, pp. 158-162, doi: 10.1007 / PL00002098

Web links

Individual evidence

  1. ↑ Alphabetical directory for the ICD-10-WHO Version 2019, Volume 3. German Institute for Medical Documentation and Information (DIMDI), Cologne, 2019, p. 810