Cutaneous papules and nodules in the diagnosis of the antiphospholipid syndrome

Abstract

Of 11 patients with primary or secondary antiphospholipid syndrome (APS), four exhibited papules or nodules on the finger, sole or leg as the initial cutaneous manifestation. Histological examination demonstrated thrombosed vessels or vessels containing organized thrombi in the dermis or in the subcutaneous fat tissue. Cutaneous papules and nodules should be recognized as skin manifestations of APS. Screening tests for antiphospholipid antibodies and lupus anticoagulant are required in patients with cutaneous papules or nodules of unknown aetiology. In cases of positive antiphospholipid antibodies and/or lupus anticoagulant, histological examination is critical in the establishment of the diagnosis of APS.

Dermatologic manifestations of the antiphospholipid syndrome: Two hundred consecutive cases

“…They were the presenting manifestations in 2.6% of the 1,000 patients with APS in the European cohort and were observed in 3.9% of these patients during the course of the disease (5). Different features have been reported, i.e., purpura, small erythematous or cyanotic lesions on the hands and feet (28), papules or nodules on the limbs, ears, neck, or thighs (29–32), and localized necrotic areas of the neck and anterior chest (33).…”

“…For other isolated skin lesions such as circumscribed necrosis or pseudovasculitis, combination therapy with low‐dose aspirin and dipyridamole has been reported to be effective in some cases (31, 32). If these lesions recur or extend despite antiplatelet treatment, anticoagulation is usually prescribed.…”

Interstitial granulomatous dermatitis with plaques associated with antiphospholipid syndrome

“…chlorpromazine, procainamide, quinidine, phenytoin or interferon) 3 . Cutaneous presentations in patients with APS include livedo reticularis, cutaneous ulcers, necrotizing vasculitis, livedo vasculitis, thrombophlebitis, painful skin nodules and anetoderma 3–5 . The immunopathogenesis and pathophysiology of APS, however, are not well characterized.…”

Chilblains and antiphospholipid antibodies: report of four cases and review of the literature

“…During further follow up of these patients, two developed systemic lupus erythematosus and one had criteria for antiphospholipid antibody syndrome. The classic skin manifestations in the antiphospholipid antibody syndrome are well known and include: livedo racemosa, splinter haemorrhages, porcelain‐white atrophic (Degos‐like) scars, thrombocytopenic purpura, acrocyanosis, distal cutaneous ischaemia, lower extremity ulceration, necrosis, cutaneous nodules and erythematous and purplish macules of the extremities 6 . Chilblains are painful or pruritic erythematous papules or plaques or erythematous swelling of fingers or toes.…”

“…Histologically, peri‐eccrine lymphocytic inflammation, dermal oedema and spongiosis are suggestive of chilblains. Vessels containing organized thrombus like in the antiphospholipid syndrome are rare but can sometimes be found in idiopathic chilblain 4–6 . In cases of chronicity, recurrence, associated signs of systemic disease or female sex, laboratory tests should be performed including a full blood count, serum creatinine level, serum protein electrophoresis, antinuclear antibodies, antidouble‐stranded DNA, anti‐Sm, anti‐RNP, anti‐SSA/Ro, anti‐SSB/La autoantibodies, cryoglobulinaemia, serum haemolytic complement and C3 and C4 components.…”