Nicolas Kluger – Livedoid vasculopathy
Table II . Differential diagnoses of livedoid vasculopathy ( LV ) ( according to Criado et al . ( 1 ))
Cutaneous vasculitis Cutaneous polyarteritis nodosa Antiphospholipid syndrome Chronic venous insufficiency Pyoderma gangrenosum Degos disease Dermatitis artefacta
Fig . 2 . Non-infiltrated permanent livedo racemosa of the hand in a patient with livedoid vasculopathy .
and intravascular thrombosis , segmental hyalinization and endothelial proliferation . There is a minimal perivascular lymphocytic infiltrate . Vasculitis features are absent ( Fig . 2 ) ( 8 ). Direct immunofluorescence is not specific , with immunoglobulin M ( IgM ) deposits , fibrin and complement depositions in blood vessels ( 6 ), and care should be taken that positivity is not misdiagnosed as skin vasculitis .
There are many diseases associated with LV ( 1 , 9 ). One should distinguish LV associated with thrombophilia from LV associated with autoimmune diseases and other conditions . The list of diseases is summarized in Table I . It is important to stress that 50 % of patients with LV have no identifiable association with a hypercoagulable condition ( 6 ). The differential diagnoses are summarized in Table II . The difficulty of differential diagnosis from cutaneous PAN should be noted ( 6 , 10 ). In addition , AB lesions can be associated with conditions other than LV , such as venous insufficiency ( Fig . 3 ).
Laboratory findings
No blood test can confirm the diagnosis of LV . However , certain tests should be performed to identify an underlying primary
Associated diseases , conditions and differential diagnoses
Table I . Conditions associated with livedoid vasculopathy ( 1 , 9 )
Thrombophilia Activated C protein resistance Prothrombin gene mutation ( G20210A ) Mutation of MTHFR ( C677T ), Hyperhomocysteinaemia Protein C deficiency Protein S deficiency Factor V Leiden mutation Antithrombin deficiency High levels of type 1 inhibitor of tissue plasminogen activator ( PAI-1 ) Increased levels of lipoprotein ( a ) Antiphospholipid antibodies : lupus anticoagulant and / or anticardiolipin antibody , anti-phosphatidylserine-prothrombin complex antibody Cryoglobulinaemia Cryofibrinogenaemia
Autoimmune and other diseases Systemic lupus erythematosus Rheumatoid arthritis Systemic scleroderma Mixed connective tissue disease Solid carcinoma Haematological malignancies
Fig . 3 . Histological findings of livedoid vasculopathy : thrombosis of the skin vessels in the upper dermis with extravasation of red blood cells but lack of leukocytoclasia and inflammation ( haematoxylin and eosin , × 4 ).
4 Educational Review
Forum for Nord Derm Ven 2018 , Vol . 23 , No . 1