Livedo: benign livedo reticularis (no tests) or livedo racemosa (initial work-up)
BioMG follows French guidelines. Adapt it to the guidelines and the tests available where you practise.
Benign livedo reticularis (fine, closed mesh, symmetrical, cold-related, isolated): no tests
- No tests if the livedo is typically physiological and the examination is normal.
Do not order
- Extensive laboratory tests for a typical, isolated, cold-related livedo reticularis: very unlikely to be useful [1].
Livedo racemosa or suspicious livedo: first tests
Laboratory tests (fasting from fat if cryoglobulins)
- State the context (livedo racemosa) and any anticoagulant treatment on the request form: type, dose, time of the last dose.
- Lupus anticoagulant (citrate tube)
- Anticardiolipin antibodies IgG and IgM
- Anti-beta-2-glycoprotein I antibodies, IgG and IgM
- FBC (platelets)
- PT, aPTT
- CRP
- Antinuclear antibodies (ANA)
- Serum creatinine with eGFR
- Urine protein:creatinine ratio (spot sample)if neededIf hypertension or abnormal renal function: antiphospholipid-associated nephropathy presents with hypertension, impaired renal function and proteinuria; the PNDS includes spot-sample proteinuria in the extension work-up of APS [2].
- Cryoglobulins (sample collected and transported at 37 °C, fasting from fat; laboratory with the necessary equipment)if neededIf purpura, acrosyndrome, leg ulcer or hepatitis C: cryoglobulinaemia causes a pathological livedo with large irregular meshes [6]. Sample collected with pre-warmed equipment at 37 °C, transported at 37 °C, patient fasting from fats for 4 to 6 hours; if these conditions cannot be met, it is better not to take the sample [6]. The Sajjan review lists it among the useful screening tests [1].
- Hepatitis C serology (if cryoglobulins positive or risk factor)if neededHCV is responsible for 40 to 60% of mixed cryoglobulinaemias [6].
12 weeks later, if an antiphospholipid antibody is positive
- If an antibody is positive: repeat it 12 weeks later, away from any inflammation.
Do not order
Sources (9)
- Sajjan VV, Lunge S, Swamy MB, Pandit AM. Livedo reticularis: a review of the literature. Indian Dermatol Online J. 2015;6(5):315-21.
- Centre de référence du lupus, syndrome des anticorps antiphospholipides et autres maladies auto-immunes rares ; centre de référence RAISE ; filière FAI²R. Protocole national de diagnostic et de soins (PNDS) : syndrome des anti-phospholipides de l'adulte et de l'enfant. Haute Autorité de santé, octobre 2022.
- Ngan V, de Menezes S, Oakley A. Livedo reticularis. DermNet (Nouvelle-Zélande), 2003, mise à jour en septembre 2016.
- Barbhaiya M, Zuily S, Naden R, et al. 2023 ACR/EULAR antiphospholipid syndrome classification criteria. Ann Rheum Dis. 2023;82(10):1258-1270 (également Arthritis Rheumatol. 2023;75(10):1687-1702).
- Arachchillage DRJ, Gomez K, Alikhan R, Anderson JAM, Lester W, Laffan M; British Society for Haematology. Addendum to British Society for Haematology Guidelines on Investigation and Management of Antiphospholipid syndrome, 2012: use of direct acting oral anticoagulants. Br J Haematol. 2020;189(2):212-215.
- Centre de référence des maladies auto-immunes systémiques rares d'Île-de-France ; filière FAI²R (coordination : Cacoub P). Protocole national de diagnostic et de soins (PNDS) : cryoglobulinémies. Haute Autorité de santé, juin 2021.
- Arachchillage DJ, Platton S, Hickey K, et al.; BSH Committee. Guidelines on the investigation and management of antiphospholipid syndrome. Br J Haematol. 2024;205(3):855-880.
- Tripodi A, Cohen H, Devreese KMJ. Lupus anticoagulant detection in anticoagulated patients. Guidance from the Scientific and Standardization Committee for lupus anticoagulant/antiphospholipid antibodies of the International Society on Thrombosis and Haemostasis. J Thromb Haemost. 2020;18(7):1569-1575.
- Lee PY, Davidson BA, Abraham RS, et al. Evaluation and Management of Deficiency of Adenosine Deaminase 2: An International Consensus Statement. JAMA Netw Open. 2023;6(5):e2315894.