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