Cutaneous manifestations of antiphospholipid syndrome

Abstract

Antiphospholipid syndrome (APS) is an acquired thrombophilic disorder in which autoantibodies are produced against a variety of phospholipids and phospholipid-binding proteins. The purpose of this article is to review cutaneous findings in patients with APS diagnosis. An overview regarding prevalence, description, pathogenesis and histopathology, are described for cutaneous manifestations of APS.

Primary antiphospholipid syndrome in a male presents with acute digital ischemia: Dramatic response to glucocorticoid

“…6 Although around 50% of patients present with various dermatological symptoms, 3.3%-7.5% of APS patients develop digital gangrene, while it is the presenting feature among only 1.9%-2.5% of individuals. 2,6,7,9 Risk factors for thrombotic events such as smoking, hypertension, oral contraceptive pills, dyslipidemia, infection, immobilization, trauma, and surgery may contribute to gangrene development. 2,10,11 Our patient was a smoker, which is a risk factor.…”

“…Gangrene can be preceded by distal erythema, cyanotic macules, or pseudo‐cellulitis resulting in necrosis and occasionally leading to amputation of the digits 7,9 . Peripheral pulses may be preserved or absent 2 . Angiography may show large or medium size vessel stenosis 9 .…”

“… 2 , 6 , 7 , 9 Risk factors for thrombotic events such as smoking, hypertension, oral contraceptive pills, dyslipidemia, infection, immobilization, trauma, and surgery may contribute to gangrene development. 2 , 10 , 11 Our patient was a smoker, which is a risk factor. Gangrene can be preceded by distal erythema, cyanotic macules, or pseudo‐cellulitis resulting in necrosis and occasionally leading to amputation of the digits.…”

“…Antiphospholipid syndrome (APS) is an autoimmune disease characterized by the presence of antiphospholipid antibodies, namely, lupus anticoagulant (LA), anticardiolipin antibodies (aCL), or antibodies to the protein cofactor‐β 2 glycoprotein I (anti‐β 2 GPI) which cause a hypercoagulable state resulting in recurrent venous, arterial or small vessel thromboembolism and/or pregnancy‐related complications 1–3 . Thrombus can be either venous (59%) or arterial (28%) or both arterial and venous (13%) 2 . It was first described by Hughes in 1983, hence also known as Hughes syndrome 4 .…”

“…1 , 2 , 3 Thrombus can be either venous (59%) or arterial (28%) or both arterial and venous (13%). 2 It was first described by Hughes in 1983, hence also known as Hughes syndrome. 4 In 1992, another subset of APS (<1%), termed catastrophic APS, was identified where multiple small vessels supplying major organs infarct within a short period of time leading to multi‐organ failure and high mortality (>50%).…”