Graham Hughes1 and Shirish Sangle2
(1)
The London Lupus Centre, London Bridge Hospital, London, UK
(2)
Louise Coote Lupus Unit, St Thomas’ Hospital, London, UK
Abstract
The commonest skin manifestations of APS are livedo reticularis and thrombotic skin ulcers.
The commonest skin manifestations of APS are livedo reticularis and thrombotic skin ulcers.
12.1 Livedo Reticularis
This is one of the most important physical signs in APS and is felt by many physicians to be an additional risk factor for the risk of APS clinical events, over and above the presence of aPL (Fig. 12.1).
Figure 12.1
Livedo reticularis in Hughes Syndrome
It has a characteristic blotchy appearance, described by one patient as “corned beef skin” and is most commonly seen on the knees, thighs, buttocks and the wrists. The livedo is a result of diminished blood flow from the deeper skin arterioles to the more superficial venules and capillaries. It is seen in a variety of conditions which affect inflow or outflow to skin vessels (including hyperviscosity syndromes). Dermatologists recognise two main forms, the pathologically significant form having an irregular, incomplete pattern (livedo racemosa). Histologically, the picture is one of obliterating vascular lesions, with no evidence of inflammatory vasculitis.
The clinical severity can vary but it is interesting to note the improvement in livedo seen in many patients with Hughes Syndrome with effective anticoagulation.
12.2 Skin Ulcers
Skin ulcers are an important feature of APS with reports of up to 30% of APS patients developing ulcers – most commonly leg ulcers. They are presumed secondary to venous thrombosis, though in many cases there is no clear antecedent history of, for example, DVT.
They vary in size from ½ cm in diameter, through to large, necrotic leg ulcers several centimetres across. Clinical experience has shown that healing in many cases, though not all, is improved with anticoagulation treatment (Fig. 12.2).
Figure 12.2
Cutaneous ulcer in Hughes Syndrome (Khamashta MA, Hughes Syndrome: Antiphospholipid Syndrome, 2nd ed., 2006, reproduced with permission from Springer
12.3 Digital Gangrene
This, a well-known complication of Hughes Syndrome, can follow either isolated arterial thrombosis or be a part of the more widespread thrombotic process seen in the catastrophic APS (Fig. 12.3).
Figure 12.3
Digital Gangrene in Hughes Syndrome, (Khamashta MA, Hughes Syndrome: Antiphospholipid Syndrome, 2nd ed., 2006, reproduced with permission from Springer (Khamashta MA, Hughes Syndrome: Antiphospholipid Syndrome, 2nd ed., 2006, reproduced with permission from Springer)
12.4 Splinters
Subungual nail-fold infarcts are a prominent feature in some cases. They can be multiple (as in infectious endocarditis) and, strikingly, can be a recurrent feature pre-menstrually in some APS patients (Fig. 12.4).
Figure 12.4
Splinter hemorrhage in a fingernail in Hughes syndrome