{
  "abstract": "Objectives Cutaneous manifestations are among the most frequent non-criteria signs of antiphospholipid syndrome (APS). They occur in up to 50% of patients and may precede thrombotic or obstetric events in 30–40% of cases. The 2023 ACR/EULAR classification criteria for APS included livedo racemosa and livedoid vasculopathy within the microcirculatory domain, emphasizing the diagnostic relevance of dermal involvement. The aim of the study was to assess the frequency and spectrum of cutaneous manifestations in APS and their associations with clinical features and antiphospholipid antibody (aPL) profiles.Methods The study included 117 patients (75 females, 42 males): 78 with primary APS (PAPS) and 39 with secondary APS (SAPS). Median age was 39.0 [33.0–47.0] years, and median disease duration 98.0 [30.0–180.0] months. All patients fulfilled the 2006 APS criteria. Twenty-eight patients (24%) had both thrombotic and obstetric events. IgG-aCL and IgG-anti-β2GPI levels were significantly higher in PAPS than SAPS (p = 0.01).Results Cutaneous lesions were observed in 64% of APS patients — in 49% of those with PAPS and 87% with SAPS. In PAPS, livedo occurred in 41%, skin ulcers in 12%, lipodermatosclerosis in 29%, and necrosis or gangrene in 2% of patients. The presence of cutaneous lesions was associated with a nearly threefold increase in arterial thrombosis and a 6.5-fold higher frequency of cerebral microangiopathy. All PAPS patients with thrombotic nephropathy or epileptiform hyperkinesia had skin involvement. In SAPS, cutaneous lesions were also seen in patients with thrombotic nephropathy, bone necrosis, and hyperkinesia. In PAPS, IgG-aCL and combined IgG-aCL/IgG-anti-β2GPI positivity were linked to an approximately 3.5-fold higher risk of livedo, while isolated IgG-anti-β2GPI positivity increased the risk by 3.8-fold. Livedo occurrence was unrelated to IgM isotypes or non-criteria aPL. No significant associations were found between livedo and aPL in SAPS. One catastrophic APS case presented with extensive necrosis (approximately 30% of body surface), being LA positive but aCL/anti-β2GPI negative.Conclusions Cutaneous involvement is common in APS and may reflect systemic microvascular injury. Livedo racemosa in PAPS was significantly associated with IgG-aCL and IgG-anti-β2GPI, supporting its inclusion as a key marker of vascular risk.",
  "authors": [
    {
      "affiliations": [
        "V. A. Nasonova Research Institute of Rheumatology, Laboratory of Thromboinflammation, Moscow, Russia"
      ],
      "name": "Anastasiia Shumilova"
    },
    {
      "affiliations": [
        "V. A. Nasonova Research Institute of Rheumatology, Laboratory of Thromboinflammation, Moscow, Russia"
      ],
      "name": "Maria Cherkasova"
    },
    {
      "affiliations": [
        "V. A. Nasonova Research Institute of Rheumatology, Laboratory of Thromboinflammation, Moscow, Russia",
        "Russian Medical Academy of Continuing Professional Education, Department of Rheumatology, Moscow, Russia"
      ],
      "name": "Aleksander Lila"
    },
    {
      "affiliations": [
        "V. A. Nasonova Research Institute of Rheumatology, Laboratory of Thromboinflammation, Moscow, Russia",
        "Russian Medical Academy of Continuing Professional Education, Department of Rheumatology, Moscow, Russia"
      ],
      "name": "Tatiana Reshetnyak"
    }
  ],
  "title": "PO:01:014 Cutaneous features and antibody profiles in primary and secondary antiphospholipid syndrome",
  "uid": "fbb4be66-23e6-5611-bc9e-2b92dad34158"
}