Livedovaskulopathie

Bei der Livedovaskulopathie kommt es durch eine Gefäßokklusion zur Ischämie mit anschließender Ulzeration der Haut. Die Erkrankung hat chronisch rezidivierende Verläufe und tritt ausschließlich an der unteren Extremität auf. Dieser Artikel beschreibt die klinischen Besonderheiten und die Therapieoptionen der Livedovaskulopathie. Die Empfehlungen stehen im Einklang mit der aktuellen S1-Leitlinie „Diagnostik und Therapie der Livedovaskulopathie“.

Abstract

Livedovasculopathy is a chronic and recurrent disease in which an insufficient perfusion of the skin leads to ischemia and subsequent ulceration. Painful, recurrent ulcerations on the lower legs are characteristic. A clinical triad consisting of livedo racemosa, ulceration and atrophie blanche is typical. The disease manifests itself exclusively in the lower extremity and especially in the malleolar region. Typically, the patients describe burning pain, which is limited to the place of manifestation does not show any seasonal accumulation. The diagnosis of LV is based on the clinical image and histology. The histological confirmation should be carried out in the acute stage of ischemia. The typical fibrin thrombi can be detected with a spindle biopsy from the peripheral area of the manifestation.

First-line therapy consists of low molecular weight heparin, direct oral anticoagulants, or intravenous immunoglobulins. The rapid diagnosis and initiation of therapy to avoid irreversible ulcerations is crucial for the long-term management.

Schlüsselwörter

Livedo racemosa - Ulzeration - Atrophie blanche - Thrombus

Keywords

livedo racemosa - ulceration - atrophie blanche - thrombus

Publikationsverlauf

Artikel online veröffentlicht:
13. April 2022

• Literatur

• 1 Feldaker M, Hines Jr EA, Kierland RR. Livedo reticularis with summer ulcerations. AMA Arch Derm 1955; 72: 31-42 DOI: 10.1001/archderm.1955.03730310033007. (PMID: 14387292)
• 2 Goerge T, Schiffmann M-L, Dissemond J. et al. S1-Leitlinie Diagnostik und Therapie der Livedovaskulopathie. J Dtsch Dermatol Ges 2021; 19 (11) 1667-1678 DOI: 10.1111/ddg.14520_g. (PMID: 34811901)
• 3 Micieli R, Alavi A. Treatment for livedoid vasculopathy: a systematic review. JAMA Dermatol 2018; 154: 193-202 DOI: 10.1001/jamadermatol.2017.4374. (PMID: 29141075)
• 4 Fritsch P, Zelger B. Livedo vasculitis. Hautarzt 1995; 46: 215-224 DOI: 10.1007/s001050050243. (PMID: 7759251)
• 5 Weishaupt C, Strolin A, Kahle B. et al. Characteristics, risk factors and treatment reality in livedoid vasculopathy – a multicentre analysis. J Eur Acad Dermatol Venereol 2019; 33 (09) 1784-1791 DOI: 10.1111/jdv.15639. (PMID: 31009111)
• 6 Goerge T. Livedoid vasculopathy. Pathogenesis, diagnosis and treatment of cutaneous infarction. Hautarzt 2011; 62: 627-634 DOI: 10.1007/s00105-011-2172-3. (PMID: 21786003)
• 7 Dodman B, Cunliffe WJ, Roberts BE. Observations on tissue fibrinolytic activity in patients with cutaneous vasculitis. Br J Dermatol 1973; 88: 231-235 DOI: 10.1111/j.1365-2133.1973.tb07539.x. (PMID: 4514551)
• 8 Weishaupt C, Strolin A, Kahle B. et al. Anticoagulation with rivaroxaban for livedoid vasculopathy (RILIVA): a multicentre, single-arm, open-label, phase 2a, proof-of-concept trial. Lancet Haematol 2016; 3: e72-e79 DOI: 10.1016/S2352-3026(15)00251-3. (PMID: 26853646)
• 9 Monshi B, Posch C, Vujic I. et al. Efficacy of intravenous immunoglobulins in livedoid vasculopathy: long-term follow-up of 11 patients. J Am Acad Dermatol 2014; 71: 738-744 DOI: 10.1016/j.jaad.2014.05.039. (PMID: 25022849)
• 10 Polo Gascón MR, de Carvalho JF, de Souza Espinel DP. et al. Quality-of-life impairment in patients with livedoid vasculopathy. J Am Acad Dermatol 2014; 71: 1024-1026 DOI: 10.1016/j.jaad.2014.06.030. (PMID: 25437971)
• 11 Hairston BR, Davis MD, Pittelkow MR. et al. Livedoid vasculopathy: further evidence for procoagulant pathogenesis. Arch Dermatol 2006; 142: 1413-1418 DOI: 10.1001/archderm.142.11.1413. (PMID: 17116831)
• 12 Vasudevan B, Neema S, Verma R. Livedoid vasculopathy: A review of pathogenesis and principles of management. Indian J Dermatol Venereol Leprol 2016; 82: 478-488 DOI: 10.4103/0378-6323.183635. (PMID: 27297279)
• 13 Marsch WC, Komatsuzaki S, Mueller A. et al. Livedoid vasculopathy: does hyperhomocysteinaemia play an aetiological role?. Eur J Dermatol 2019; 29: 287-293 DOI: 10.1684/ejd.2019.3554. (PMID: 31389788)
• 14 Hairston BR, Davis MD, Gibson LE. et al. Treatment of livedoid vasculopathy with low-molecular-weight heparin: report of 2 cases. Arch Dermatol 2003; 139: 987-990 DOI: 10.1001/archderm.139.8.987. (PMID: 12925383)
• 15 Goerge T, Weishaupt C, Metze D. et al. Livedoid vasculopathy in a pediatric patient with elevated lipoprotein(a) levels: prompt response to continuous low-molecular-weight heparin. Arch Dermatol 2010; 146: 927-928
• 16 Schobess R, During C, Bidlingmaier C. et al. Long-term safety and efficacy data on childhood venous thrombosis treated with a low molecular weight heparin: an open-label pilot study of once-daily versus twice-daily enoxaparin administration. Haematologica 2006; 91: 1701-1704 (PMID: 17145610)
• 17 Furukawa F, Mizawa M, Makino T. et al. Efficacy of new low-dose oral anticoagulants in recalcitrant livedoid vasculopathy. BMJ Case Rep 2017; DOI: 10.1136/bcr-2017-219943. (PMID: 28739563)
• 18 Sawada T, Suehiro M. Dabigatran in the management of livedoid vasculopathy. Clin Exp Dermatol 2017; 42: 237-239 DOI: 10.1111/ced.13022. (PMID: 28052437)
• 19 Pengo V, Denas G, Zoppellaro G. Rivaroxaban vs warfarin in high-risk patients with antiphospholipid syndrome. Blood 2018; 132: 1365-1371 DOI: 10.1182/blood-2018-04-848333. (PMID: 30002145)
• 20 Kofler K, Strölin A, Geiger V. et al. Intravenous Immunoglobulin Therapy in Livedoid Vasculopathy: Retrospective Observation of Clinical Outcome and Patient’s Activity Level. J Cutan Med Surg 2021; DOI: 10.1177/12034754211003525.
• 21 Ravat FE, Evans AV, Russell-Jones R. Response of livedoid vasculitis to intravenous immunoglobulin. Br J Dermatol 2002; 147: 166-169 DOI: 10.1046/j.1365-2133.2002.04755.x. (PMID: 12100203)
• 22 Ramírez E, Romero-Garrido JA, López-Granados E. et al. Symptomatic thromboembolic events in patients treated with intravenous-immunoglobulins: results from a retrospective cohort study. Thromb Res 2014; 133: 1045-1051 DOI: 10.1016/j.thromres.2014.03.046. (PMID: 24731561)
• 23 Kerk N, Goerge T. Livedoid vasculopathy – current aspects of diagnosis and treatment of cutaneous infarction. J Dtsch Dermatol Ges 2013; 11: 407-410 DOI: 10.1111/ddg.12064. (PMID: 23437985)