Primary anetoderma: a cutaneous sign of antiphospholipid antibodies

Abstract

Although a few reports in recent years have suggested that patients with antiphospholipid antibodies (aPL) are proneto developingprimaryanetoderma(PA), it is still unclearhowoften aPL are detectedin unselected PA patients. We studied nine consecutive PA patients for the presence of autoimmune antibodies and disorders in general and the presence of aPL in particular. Six of the nine patients had clinical evidence of associated autoimmune disorders (Graves’disease and autoimmune haemolysis in one, systemic scleroderma in one, Hashimoto’s thyroiditisin one, alopecia areata in one) and/or signs of hypercoagulability (recurrent fetal loss in two, recurrent strokes in one, recurrent deep vein thrombosis in one). In four of these six patients the onset of PA preceded these signs. Positive aPL was foundin all: anticardiolipin(aCL) in six, anti-β₂-glycoprotein-I(aβ₂ GPI) in six and lupusanticoagulant (LAC) in four. The most frequent isotype was IgA. Among other autoantibodies found the most frequentlywas antinuclearantibodies.Four of theninepatientsfulfilled thecriteriaforantiphospholipid syndrome (APS). It is concluded that PA is an important cutaneous sign for autoimmune disorders in general and the presence of aPL in particular. Hence, the work-up of these patients should include testingfor LAC as well as forall differentisotypesof aCL andaβ₂ GPI. We recommendthat PA be added to the list of the cutaneous manifestations of APS.