Cutaneous Necrosis Associated with the Lupus Anticoagulant

Abstract

Extensive cutaneous necrosis of the limbs with sudden onset was observed in 3 women suffering from benign systemic lupus erythematosus. All 3 had a circulating anticoagulant and a positive venereal diseases reference laboratory test without anticardiolipin antibodies.They were successfully treated with pulse methylprednisolone therapy and plasmapheresis. The necrosis-lupus anticoagulant relationship is discussed due to the absence of another possible etiology for cutaneous necrosis and the latter’s histological aspect, i.e. thromboses of the dermal and hypodermal vessels without vasculitis. This cutaneous necrosis resembles that observed in congenital protein C deficiencies and favors the hypothesis of interaction between the lupus anticoagulant and the protein C-S complex.

Diffuse skin necrosis in a patient with an anti‐endothelial cell protein C receptor autoantibody which blocks protein C activation

“…It is described in patients developing heparindependent, platelet-activating antibodies [10] in patients with antiphospholipid antibodies [11]-sometimes associated with an acquired protein S deficiency [12]-and during severe sepsis, such as meningococcemia, known to induce a severe protein C consumption [13].…”

Cutaneous necrosis in a young woman

“…Cutaneous necrosis secondary to intravascular thrombosis of small dermal vessels is a rare association with antiphospholipid syndrome. Many reported cases suggest that it is far more common in women 2 . Approximately 40% of patients will present with a cutaneous manifestation as the first indication of this disease, with a marked number of patients concurrently developing multiorgan involvement 3 .…”

Cutaneous papules and nodules in the diagnosis of the antiphospholipid syndrome

“…There have been numerous reports documenting the cutaneous changes in APS. Common cutaneous manifestations include livedo reticularis (racemosa), 9 –11 thrombophlebitis 12 and cutaneous ulcers, gangrene or necrosis 13 –18 . Alegre et al 12 .…”