SNEDDON'S SYNDROME

By Gary M. White, MD


Sneddon's Syndrome is a rare vasculopathy characterized by livedo racemosa and CNS symptoms (e.g. transient ischemic attack, stroke). The typical patient is a woman 20-40 years of age.

Clinical

A livedo pattern of redness or erythema is present (i.e., livedo racemosa). Headache and dizziness are common and progressive cognitive impairment may develop over time. The kidneys, heart, and eyes may be affected. Other causes of livedo racemosa should be excluded.

Workup

Workup should focus on any conditions that cause a vasculopathy, e.g., lupus, antiphospholipid syndrome, etc. Blood work may include antinuclear antibodies, anti-dsDNA, anticardiolipin and antineutrophil cytoplasmic antibodies, lupus anticoagulant, protein C, protein S, etc. See also livedo reticularis. The patient should be questioned about any headaches, vertigo, seizures and cognitive impairment. A skin biopsy should be generous and obtained from the center of an annular livedo segment.

Treatment

It is important for patients to avoid smoking, estrogen-containing OCPs or pregnancy, and obesity. Usually treatment with steroids, immunosuppressive agents, and anticoagulants is done by the non-dermatologist.

References

Internat Journal Derm Ven Lep 2012;78;407

A woman in her 40's presented with a 4-hour history of ataxia and left sided hemidysesthesia. Annular patches of violaceous erythema were noted on the trunk and extremities and had been present for 2 years. Brain MRI showed acute changes and old stroke residues [JAMA Derm 2018;93].

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