[Sneddon syndrome: vasculitis or thrombotic disorder?].
BACKGROUND
Livedo reticularis generalisata (LR) in combination with affection of CNS is referred to as Sneddon's syndrome (SNS). Latest data suggest chronic progressive systemic disorder with occlusion of small and medium sized vessels (e.g., cutis, brain, kidneys, heart, eyes). No conclusive etiology is known, though there are correlations to the antiphospholipid syndrome, systemic secondary vasculitis and coagulopathies. Hereditary and toxic factors seem to play a role in pathogenesis in some cases.


METHODS
Diagnostic procedure and clinical course of a 56-year-old woman with dementia and hemiparesis proceeded by LR is reported. MRI-, SPECT- and TCD-findings were congruent with diffuse ischemic lesions of the brain due to affection of small- and medium-sized vessels. Histopathological specimens of the brain, meninges and cutis were non diagnostic. Some laboratory findings suggested vasculitis as an underlying cause. LR improved under immunosuppressive therapy with prednisolone and azathioprin.


CONCLUSIONS
SNS does not seem to be a nosological entity. A differentiation between primary (idiopathic) and secondary SNS is useful for different therapeutical approaches.
Zipper
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Neurologische Klinik, St.-Katharinen-Krankenhaus.
Lambert
S
S
Seemann
W R
WR
Baer
U
U
Schlisske
K
K
D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D001241:Aspirin / Q000627:therapeutic use; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology* / Q000503:physiopathology; D003704:Dementia / Q000209:etiology; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D017445:Skin Diseases, Vascular / Q000209:etiology; D018860:Sneddon Syndrome / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000503:physiopathology*; D013256:Steroids; D015899:Tomography, Emission-Computed, Single-Photon; D020293:Vasculitis, Central Nervous System / Q000503:physiopathology
D000893:Anti-Inflammatory Agents; D000894:Anti-Inflammatory Agents, Non-Steroidal; D013256:Steroids; D001241:Aspirin