Forum for Nordic Dermato-Venereology Nr 1, 2018 | Page 7

Nicolas Kluger – Livedoid vasculopathy
Table III. Suggested laboratory tests in cases of livedoid
vasculopathy (LV)
Full blood count
Inherited and acquired thrombophilia investigations (cf. Table I):
prothrombin time, bleeding time, activated partial thromboplastin
time, ...
CRP, fibrinogen, serum protein electrophoresis
ANA, anti-DNA antibody, ANCA, RF, CH50, C3, C4
Cryoglobulinaemia
Antiphospholipid antibodies
diagnosis (Table III) (9). In addition to those tests, Doppler
imaging has to be performed to rule out venous insufficiency.
Management
The management of LV is notoriously challenging and dif-
ficult. There is currently no treatment consensus (11). LV is
also a self-remitting disease that can improve on its own (11).
Pain management is crucial and must include the treatment
of nociceptive and neuropathic pain components. Concom-
itant chronic venous disease will be managed by adequate
compression therapy in the absence of arterial disease and
adapted wound dressings (6).
Despite the lack of detection of prothrombotic abnormali-
ties in many patients, it is likely that a thrombotic process
is involved in the pathogenesis of LV, and thus it is widely
accepted that the main treatment is antithrombotic. Platelet
aggregation inhibitors can be used, such as acetylsalicylic
acid, dipyridamole, or thienopyridine (clopidogrel, ticlopidine
hydrochloride), but for some (12), treatment with antiplatelet
drugs rarely results in complete remission. Anticoagulant ther-
apies with low-molecular-weight heparin at a curative dose
followed by oral vitamin K antagonists (warfarin) (12) or oral
rivaroxaban, a Factor Xa inhibitor, have demonstrated efficacy
(13). Corticosteroids may be used in the case of underlying
connective tissue diseases (11). Other treatments are summa-
Table IV. Possible treatments for livedoid vasculopathy (LV)
(1, 11)
Platelet aggregation inhibitors: acetylsalicylic acid, dipyridamole,
thienopyridine (clopidogrel, ticlopidine hydrochloride)
Anticoagulants: low-molecular-weight heparin, oral vitamin K
antagonists, factor Xa inhibitors
IV methylprednisolone + pentoxifylline
Vasodilators: nifedipine, cilostazol (phosphodiesterase III inhibitor),
nicotinic acid
Hemorrheologic drugs: pentoxifylline, buflomedil hydrochloride
PUVA
Low-molecular-weight dextran
Rituximab
Miscellaneous: IV immunoglobulins, cyclosporine A, hyperbaric
oxygen therapy, PGI2 analogue IV, tissue plasminogen activator
Forum for Nord Derm Ven 2018, Vol. 23, No. 1
Fig. 4. Atrophie blanche (AB) and small ulcers (currently healing) in a
33-year-old patient with a known history of chronic venous insufficien-
cy and no prothrombotic abnormalities. No biopsy was taken and the
ulcers were not painful according to the patient. The diagnosis currently
remains open as to whether AB is related to venous insufficiency or
possible livedoid vasculopathy.
rized in Table IV. They may be limited by their costs, such as
intravenous immunoglobulins. Many patients are treated with
a combination of various regimens.
References
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atic review. JAMA Dermatol 2017 Nov 15 [Epub ahead of print].
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Arch Dermatol 2004; 140: 1011.
13. Weishaupt C, Strölin A, Kahle B, Kreuter A, Schneider SW, Gerss J,
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.
E ducational R eview
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