Images in Clinical Medicine

Angiomatosis Associated with Sneddon's Syndrome

Josef G. Heckmann, M.D., and M. Lüfti, M.D.

N Engl J Med 2004; 350:e11March 18, 2004

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A 34-year-old woman was admitted for acute right hemiparesis, which resolved within 24 hours. She had no history of hypertension, diabetes, migraine, or smoking. Since early childhood, she had had widespread cutaneous, mottled-purple discoloration on her face (Panel A) and other parts of her body, which had been diagnosed as systemic angiomatosis. The result of a test for antiphospholipid antibodies was positive (47.4 IgG phospholipid units per milliliter [normal range, <20]). T₂-weighted magnetic resonance images revealed multiple periventricular lesions within the white matter of the brain (Panel B). Examination of a skin-biopsy specimen showed a thickened vessel wall with intimal proliferation and thrombotic occlusion of subcutaneous arterioles — findings consistent with the diagnosis of angiomatosis and Sneddon's syndrome.

Sneddon's syndrome is a rare entity characterized by livedo reticularis and cerebrovascular lesions that are probably due to progressive occlusive and noninflammatory arteriopathy. Its various manifestations include angiomatosis. The best way to confirm the diagnosis is by skin biopsy, but in addition, the results of tests for antiphospholipid antibodies are often positive.

Josef G. Heckmann, M.D.
M. Lüfti, M.D.
University of Erlangen–Nuremberg, 9105 Erlangen, Germany

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