Hughes Syndrome: Highways and Byways by Graham Hughes & Munther A. Khamashta

Hughes Syndrome: Highways and Byways by Graham Hughes & Munther A. Khamashta

Author:Graham Hughes & Munther A. Khamashta
Language: eng
Format: epub
Publisher: Springer London, London


10.1 Livedo Reticularis

This striking reticular patterned skin feature (known as ‘corned beef’ skin by a number of our patients) is an important physical sign in Hughes syndrome, most commonly sited on the limbs (Fig. 10.1). Histologically, the picture is one of noninflammatory obliterating small vessel thrombosis.

In our experience, livedo appears to add an increased risk factor for thrombosis – especially arterial thrombosis and stroke. It is also clinically associated with labile hypertension, presumably reflecting a more widespread vasculopathy.

In 1965, Sneddon reported a small series of patients with livedo and stroke. Subsequent studies have shown a percentage of patients with Sneddon’s syndrome being aPL positive.

For the clinician investigating a patient suspected of APS, the finding of livedo, while not specific, is a strong clue.

Figure 10.1Livedo reticularis. Classical ‘blotchy’ (‘corned beef’) skin – an important clinical sign in a number of patients with Hughes syndrome



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