Antiphospholipid Antibodies and Anetoderma: Are They Associated?

Abstract

Background: Macular atrophy or anetoderma is a rare skin disease of unknown pathogenesis, characterised by wrinkled or flaccid skin. Objective: The finding of anetoderma in 5 patients followed up because of false-positive seroreactions of syphilis prompted us to study the occurrence of antiphospholipid (aPL) antibodies in anetoderma. Methods: 14 unselected patients with primary anetoderma (PA) were collected from hospital records and clinical, immunological and histological findings were compared in the two patient groups. Results: Of the 5 patients, 3 fulfilled the criteria for antiphospholipid syndrome. In two cases, it was secondary to systemic lupus erythematosus (SLE). Of the 14 PA patients 1 had aPL antibodies and 4 had borrelia antibodies. Two patients had thyroid antibodies; 1 of them developed SLE. In several biopsy specimens, microthromboses were seen in both patient groups. Conclusion: On the basis of this study and our previous findings, it seems that anetoderma is more often associated with aPL-positive SLE or lupus-like disease than with aPL-negative disease. Immunological mechanisms play an important role in both primary and secondary anetoderma. The meaning of false-positive serological tests for syphilis or borrelia and aPL antibodies is not clear, but they may be reacting to some still unidentified antigen. Probably, various systemic as well as local inflammatory and non-inflammatory processes, e.g. microthromboses, can trigger anetoderma via still unknown pathomechanisms.

Subcutaneous Granuloma pyogenicum in Patients with Antiphospholipid Antibodies

“…Antiphospholipid antibodies have been reported to associate with several cutaneous diseases in which vascular abnormalities seem to play a major part. Recently, nonvascular, cutaneous diseases including melanoma and anetoderma have been reported in patients with antiphospholipid antibodies [6, 7, 8]. To our knowledge, these are the first reported cases of subcutaneous GP associated with antiphospholipid antibodies.…”

Anetoderma in a Systemic Lupus erythematosus Patient with Anti-PCNA and Antiphospholipid Antibodies

“…Several studies reveal that this skin disorder is more frequent in patients with lupus erythematosus who have these antibodies than in patients with lupus erythematosus lacking them [10, 11, 12, 13]. This fact suggests a closer relationship of anetoderma with antiphospholipid antibodies and antiphospholipid syndrome than with lupus erythematosus itself.…”

Primary Anetoderma and Antiphospholipid Antibodies—Review of the Literature

“…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.…”

“…Stephansson et al were the first to demonstrate that patients with aPL with or without SLE, are prone to develop PA [27,29]. In 1991, they found that 5 out of 33 patients with SLE and positive LAC had PA, whereas among 37 SLE patients who were LAC-negative, none had PA [27].…”

“…In 1991, they found that 5 out of 33 patients with SLE and positive LAC had PA, whereas among 37 SLE patients who were LAC-negative, none had PA [27]. They later described five patients testing falsepositive for syphilis who developed PA during long-term follow-up: three eventually fulfilled the criteria for APS, two of them having SLE, and the third a lupus-like disease [29]. They concluded that PA is more often associated with aPL-positive SLE or aPL-positive lupus-like disease than with aPL-negative disease.…”