Generalized racemose livedo with cerebrovascular lesions (Sneddon syndrome): an occlusive arteriolopathy due to proliferation and migration of medial smooth muscle cells

Marsch, Wolfgang Ch.; Muckelmann, R

doi:10.1111/j.1365-2133.1985.tb02341.x

Cited by 53 publications

(25 citation statements)

References 12 publications

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“…Symptoms are recurrent calcium oxalate nephrolithiasis and nephrocalcinosis, leading to chronic renal failure and death from uraemia. Skin biopsy demonstrates numerous characteristic elongate to diamond-shaped, radially oriented, pale yellow translucent oxalate crystals within the vessels, and vessel walls of the subcutaneous fat that were strongly birefringent under polarised light [58,80]. Also caused by arterial occlusion by embolising material is livedo racemosa in arterial myxoma [104].…”

Section: ■ Other Diseasesmentioning

confidence: 97%

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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: ■ Other Diseasesmentioning

confidence: 97%

“…Another rare disease presenting with livedo racemosa is hyperoxaluria [58,80]. Symptoms are recurrent calcium oxalate nephrolithiasis and nephrocalcinosis, leading to chronic renal failure and death from uraemia.…”

Section: ■ Other Diseasesmentioning

confidence: 99%

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

2005

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“…The cerebrovascular insults originally described by Sneddon were of limited nature, often leaving little or no residual disability. On the other hand, there have been reports of several cases with livedo and progressive cerebral lesions [10][11][12][13][14][15] or livedo combined with severe cerebral deficit [2,16,17], Histopathological findings in biopsies of cutaneous lesions in Sneddon's syndrome may be very discrete and nonspecific [3,[14][15][16]; however, they have been variously described as endarteritis obliterans [2,24], endotheliosis of arterioles in the reticular dermis with occasional thrombo sis, focal segmental intimal hyperplasia [6,25] with prolifer ation and migration of medial smooth muscle cells through a discontinuous tunica elastica interna in the ascending arterioles of the upper subcutis or reticular dermis [23). Common to all these descriptions is a noninflammatory thickening of the vessel wall with eventual occlusion of the lumen, however without vasculitis.…”

Section: Discussionmentioning

confidence: 99%

Sneddon’s Syndrome: Generalized Livedo reticularis and Cerebrovascular Disease

Devos¹,

Bulcke²,

Degreef³

et al. 1992

Dermatology

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We report two cases of Sneedon’s syndrome. Both cases had widespread livedo reticularis with repeated cerebrovascular accidents without persistent neurological deficit. In one case, hemostatic examination revealed an imbalance of plas-minogen activator-inhibitor values, possibly related to the thrombogenic propensity of the syndrome. Treatment with acetylsalicylic acid led to normalization of hemostatic parameters and resulted in a symptom-free period of more than 10 months. The importance of hemostatic screening in patients with Sneddon’s syndrome is discussed.

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“…1 Because vessel intimal endothelial and medial smooth muscle proliferations are the hallmarks of skin biopsy in SS, transposition of such a picture to the retinal vasculature may explain the angiographic staining and leakage preferentially located at arterial bifurcations and the arteriovenous crossing ( Fig. 2, C, E, and F).…”

Section: Discussionmentioning

confidence: 95%

“…1 Visual involvement in SS has been rarely described and includes homonymous hemianopia secondary to cerebral infarction, 2 bilateral central artery occlusion, 3 and peripheral retinal neovascularization. 4 We describe a 48-year-old woman with monocular relapsing vitreous hemorrhage (VH) secondary to retinal neovascularization and diffuse arterial occlusion.…”

mentioning

confidence: 99%

Diffuse Retinal Arterial Occlusion, Neovascularization, and Vitreous Hemorrhage as the Presenting Sign of Sneddon Syndrome: A 7.5-YEAR Angiographic Follow-Up

Rossi¹,

Pascarella²,

Lesnoni³

et al. 2007

RETINAL Cases &Amp; Brief Reports

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The authors describe a case of Sneddon syndrome presenting with vitreous hemorrhage and describe 7 years of follow-up with increasingly severe retinal vaso-occlusive events.S neddon syndrome (SS) is a rare disease that was first described in 1965; it is characterized by the association of cutaneous purple lacelike rash (livedo reticularis or racemosa), ischemic cerebrovascular events, and labile hypertension. 1 Visual involvement in SS has been rarely described and includes homonymous hemianopia secondary to cerebral infarction, 2 bilateral central artery occlusion, 3 and peripheral retinal neovascularization. 4 We describe a 48-year-old woman with monocular relapsing vitreous hemorrhage (VH) secondary to retinal neovascularization and diffuse arterial occlusion. To our knowledge, this is the first report of relapsing VH requiring pars plana vitrectomy as a presenting sign of SS; correlation between a 7.5-year angiographic follow-up and systemic disease progression is provided. Case ReportIn September 1998, a 48-year-old woman presented complaining of floaters and loss of vision in her left eye over the past 20 days. At presentation, visual acuity was 20/20 in the right eye and 20/400 in the left eye; slit-lamp examination of both eyes revealed clear cornea with deep and quiet anterior chamber and minimal nuclear sclerosis. Indirect ophthalmoscopy of the right eye showed mild vitreous syneresis with no cells and signs of peripheral retinal arteriolar occlusion at 4 o'clock. This analysis of the left eye showed moderate VH with some subhyaloidal heme, scattered epiretinal hemorrhages, neovascularization of the optic disk, arterial occlusion with signs of perivascular sheathing, and retinal edema superotemporally to the fovea. Fluorescein angiography (FA) revealed delayed choroidal filling inferior to the optic disk in the right eye (Fig. 1, A and B) and arterial occlusion and retinal edema superior to the fovea and some blocking due to vitreous and retinal heme in the left eye ( Fig. 2A). Early frames showed arterial filling defects, macular ischemia, and blocking due to retinal edema (Fig. 2B), while equatorial arteriovenous frames (Fig. 2, C, D, and F) disclosed vessel nonperfusion associated with staining and leakage. Late frames (Fig. 2E) showed optic nerve leakage due to neovascularization of the optic disk. Twenty days later, FA of the right eye demonstrated a filling defect of an arterial branch located 0.5 disk diameter inferior to the disk (Fig. 3, A and B). FA of the left eye had not changed substantially. Visual fields were full.The patient underwent extensive systemic evaluation and blood testing that revealed antiphospholipid antibodies present as anticardiolipin IgG and antinuclear and anti-smooth muscle antibodies well over the normal limits. Tests for rheumatoid factor and lupus anticoagulant were negative. Blood cell count was unremarkable except for thrombocytopenia (platelet count, 112,000/mL). Testing for thrombophilic conditions also was done, including screening for factors II, Leiden, and AT...

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Generalized racemose livedo with cerebrovascular lesions (Sneddon syndrome): an occlusive arteriolopathy due to proliferation and migration of medial smooth muscle cells

Cited by 53 publications

References 12 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

Sneddon’s Syndrome: Generalized Livedo reticularis and Cerebrovascular Disease

Diffuse Retinal Arterial Occlusion, Neovascularization, and Vitreous Hemorrhage as the Presenting Sign of Sneddon Syndrome: A 7.5-YEAR Angiographic Follow-Up

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