Sneddon-Syndrom: Vaskulitis oder thrombotische Vaskulopathie?

Zipper, Stephan G.; Lambert, Sybille; Seemann, Wolf-Rainer; Baer, U; Schlißke, Klaus

doi:10.1007/pl00002098

Cited by 6 publications

(7 citation statements)

References 11 publications

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“…Boortz-Marx et al presented a case with granulomatous leptomeningeal infiltrations, although chronic fibrotic changes were predominant [15]. Some authors have described an evolving vascular lesion in cases of SS, including an initial endotheliosis with infiltration of inflammatory cells from the lumen [15,93,110,111]. In aplAB-negative cases, some authors have suggested a primary inflammatory process [93], though a number of studies have not shown inflammatory changes on skin and brain biopsy [43], and the poor efficacy of immunosuppressive therapies argues against a primary inflammatory vascular process in SS [29].…”

Section: ■ Sneddon's Syndrome (Ss)mentioning

confidence: 95%

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The spectrum of differential diagnosis in neurological patients with livedo reticularis and livedo racemosa

2005

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Livedo is a cutaneous sign of striking violaceous netlike patterned erythema of the skin. This dermatological phenomenon is of special interest in the differential diagnosis in neurological patients. In 1907 Ehrmann distinguished two different patterns of livedo: the pathological livedo racemosa and the physiological livedo reticularis. Despite important clinical differences, in the English language literature the heading livedo reticularis is still used for all types of livedo. A literature review about the spectrum of differential diagnosis in patients with livedo reticularis (especially cutis marmorata and amantadine-induced livedo reticularis) and livedo racemosa (especially Sneddon's syndrome, Divry-van Bogaert syndrome, systemic lupus erythematosus, antiphospholipid antibody syndrome, polyarteritis nodosa, cholesterol embolization syndrome, livedoid vasculopathy and haematological diseases) is provided.

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Section: ■ Sneddon's Syndrome (Ss)mentioning

confidence: 95%

“…The fishnet of the livedo seems to be larger in aplAB-negative patients, who therefore frequently had obvious and sometimes troublesome skin involvement. This fact might explain the low prevalence of aplAB in dermatological series of SS [30,110]. Various prothrombotic abnormalities have also been reported.…”

Section: ■ Sneddon's Syndrome (Ss)mentioning

confidence: 96%

The spectrum of differential diagnosis in neurological patients with livedo reticularis and livedo racemosa

2005

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“…The use of corticosteroids and immunosuppressives without antithrombotic agents seems to be deleterious as noted in 6 previously reported patients [4, 26]. A temporary improvement with high-dose corticosteroids and aspirin has been reported [18]. …”

Section: Discussionmentioning

confidence: 78%

“…Not all studies agree on the validity of brain biopsy in diagnosing SNS. A recent study by Zipper et al [18]reported inconclusive results for brain biopsy. However, brain biopsy might not have been carried out by an experienced examiner, and therefore the biopsy might have missed the relevant area.…”

Section: Discussionmentioning

confidence: 99%

Therapy of Sneddon Syndrome

et al. 2002

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We report the case of a young woman with progressive cognitive decline and epilepsy. She showed ischemic cerebrovascular disease and proximal livedo racemosa. Antiphospholipid antibody (aPL) could not be detected and there were no microemboli on continuous transcranial Doppler ultrasonography monitoring. Histology of cerebral vessels showed intimal hyperplasia in small leptomeningeal venous vessels and micronecrosis of grey and white matter. We subsequently made the diagnosis of aPL-negative Sneddon Syndrome (SNS). Anticoagulation with warfarin could not be initiated because of a drug-resistant epilepsy with the risk of falls and subsequent bleeding; immunosuppression with steroids and azathioprine was ineffective, as was initial antiplatelet therapy with clopidogrel alone. However, when we intensified antiplatelet therapy by combining clopidogrel and ASS, a slowing of disease progression, as assessed by neuropsychological testing and magnetic resonance imaging, was noted on a follow-up after 6 months. Therapeutic options in SNS in both aPL-positive and aPL-negative patients with SNS are discussed.

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“…1,2 Unklar ist, ob histologisch beschriebene entzündliche Vorgänge an der Gefäßwand ursächlich Bedeutung besitzen. 3 Infolge thrombotischer Ereignisse können zudem Funktionsstörungen im zentralen Nervensystem resultieren, die sich in Form motorischer Defizite, kognitiver Beeinträchtigung, dem Auftreten transitorischer ischämischer Attacken (TIA) sowie im peripheren Nervensystem als Polyneuropathie manifestieren. 4 Das SS tritt meistens sporadisch auf, allerdings wurde auch über eine familiäre Häufung berichtet, so dass eine genetische Disposition vermutet wird und Kandidatengene identifiziert wurden.…”

Section: Introductionunclassified

Kombinationstherapie des Sneddon‐Syndroms zur Senkung der Inzidenz zerebrovaskulärer Komplikationen

Narwutsch,

Wohlrab,

Sperfeld

et al. 2024

J Deutsche Derma Gesell

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ZusammenfassungHintergrundDas Sneddon‐Syndrom wird als okkludierende Vaskulopathie verstanden, die an der Haut durch eine generalisierte Livedo racemosa und im zentralen Nervensystem durch transitorische ischämische Attacken, Schlaganfälle und kognitive oder motorische Defizite klinisch evident wird. Zur Therapie werden Thrombozytenaggregationshemmer oder Antikoagulanzien empfohlen. Aufgrund der begrenzten therapeutischen Wirksamkeit und der daraus resultierenden schwerwiegenden Komplikationen schlagen wir eine Kombinationstherapie mit zusätzlichen Infusionszyklen von Alprostadil und Captopril vor und berichten über erste Langzeitergebnisse.Patienten und MethodikAlle Patienten unserer Klinik mit primärem Sneddon‐Syndrom unter Kombinationstherapie wurden im Zeitraum von 1995 bis 2020 in eine systematische retrospektive Analyse einbezogen. Die Auswertung des therapeutischen Outcomes erfolgte mittels deskriptiver Statistik im Vergleich zu historischen Kontrollgruppen, die eine Monotherapie erhalten hatten. Zudem haben wir die Ereignisrate von Komplikationen bei Unterbrechung der Kombinationstherapie analysiert.ErgebnisseWährend des Beobachtungszeitraums der Kombinationstherapie von 99,7 Patientenjahren ereignete sich keine transitorische ischämische Attacke und die Schlaganfallrate sank auf 0,02 pro Patientenjahr. Im Vergleich lagen bei den historischen Kontrollen die Raten von transitorischen ischämischen Attacken und Schlaganfällen zwischen 0,08 und 0,035 pro Patientenjahr. Nach Unterbrechung der Alprostadil‐Therapie traten bei drei Patientinnen acht Ereignisse auf.SchlussfolgerungenDie Kombinationstherapie reduziert langfristig die Inzidenz von ischämischen Ereignissen bei Patienten mit primärem Sneddon‐Syndrom.

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Sneddon-Syndrom: Vaskulitis oder thrombotische Vaskulopathie?

Abstract: SNS does not seem to be a nosological entity. A differentiation between primary (idiopathic) and secondary SNS is useful for different therapeutical approaches.

Cited by 6 publications

References 11 publications

The spectrum of differential diagnosis in neurological patients with livedo reticularis and livedo racemosa

The spectrum of differential diagnosis in neurological patients with livedo reticularis and livedo racemosa

Therapy of Sneddon Syndrome

Kombinationstherapie des Sneddon‐Syndroms zur Senkung der Inzidenz zerebrovaskulärer Komplikationen

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