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 prone to developing primary anetoderma (PA), it is still unclear how often aPL are detected in 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 thyroiditis in one, alopecia areata in one) and/or signs of hypercoagulability (recurrent fetal loss in two, recurrent stokes in one, recurrent deep vein thrombosis in one). In four ofthese six patients the onset of PA preceded these signs. Positive aPL was found in all: anticardiolipin (aCL) in six, anti-beta2-glycoprotein-I (a(beta)2GPI) in six and lupus anticoagulant (LAC) in four. The most frequent isotype was IgA. Among other autoantibodies found the most frequently was antinuclear antibodies. Four ofthe nine patients fulfilled the criteria for antiphospholipid 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 testing for LAC as well as for all different isotypes ofaCL and a(beta)2GPI. We recommend that PA be added to the list of the cutaneous manifestations of APS.

Primary Anetoderma and Antiphospholipid Antibodies—Review of the Literature

“…However, the specific isotypes tested were not given. Four of nine PA patients in each of these two studies [36,38] fulfilled the criteria for the diagnosis of APS; in one, it was associated with SLE [38]. In two of our patients [36], the PA was the first clinical sign, preceding by many years the classical signs of APS.…”

“…This, together with the other autoimmune abnormalities that appeared many years after the onset of PA, emphasizes the need for long-term follow-up in PA patients [36].…”

“…Up to now, the literature contains reports of 35 PA patients demonstrated to have aPL, whether as an isolated laboratory finding (LAC and/or aCL, and/or aβ2GPI), as part of APS, or in conjunction with other clinical and/or laboratory signs of autoimmunity, including SLE. [26][27][28][29][30][31][32][33][34][35][36][37][38][39][40][41]. The rapid growth in the number of descriptions of such patients in the literature, from 13 patients until 2000 [26][27][28][29][30][31] to almost double since then, i.e., 22 patients [32][33][34][35][36][37][38][39][40][41], is a reflection of the increased awareness of this association among clinicians.…”

“…Thus, the actual prevalence of aPL in PA remained unclear. In our 2003 study [36] comprising nine unselected PA patients (five of whom were included in the 1992 study), the investigation included not only VDRL, LAC, and aCL isotypes G and M, but also isotype A and aβ2GPI. Positive aPL was found in all, mostly of IgA isotype (aCL and/or aβ2GPI).…”

“…Positive aPL was found in all, mostly of IgA isotype (aCL and/or aβ2GPI). We therefore suggested that the work-up of these patients should include testing for all different types of aCL and aβ2GPI [36]. In this context, an association between elevated levels of aPL IgA (aCL or aβ2GPI) and some of the cutaneous complications of APS, such as skin ulcers, has been reported [42].…”