Case Reports in Medicine Table 2: Some of the conditions associated with LV. Autoimmune diseases Systemic lupus erythematosus and antiphospholipid syndrome Rheumatoid arthritis Polyarteritis nodosa Systemic scleroderma Mixed connective tissue disease Procoagulant states Prothrombin G20210A Factor V leiden Protein C/S deﬁciency Hyperhomocysteinemia PAI 4G/4G homozygosity ulcers and secondary skin changes such as atrophic blanche can be the result of another disorder such as chronic venous insuﬃciency, vasculitis, pyoderma ganrenosum, and so forth [1]. Some of the conditions associated with LV are presented in Table 2. The pathophysiology of LV is not clear; however, it is believed that thrombosis has a putative role, whereas inﬂam- mation is believed to play a minor role [11]. Several case reports and observations pointed toward a strong association between livedoid vasculopathy and prothrombotic states. These reports will be discussed brieﬂy. Baccard et al. reported a case of LV in a patient with protein C deﬁciency, which is a vitamin-K-dependent intrinsic anticoagulant factor [12]. They reported a 65-year- old Caucasian female as in our case without signiﬁcant his- tory of thrombotic events. Interestingly, heparin, colchicine, aspirin plus dipyridamole therapy failed to improve skin ulceration in this patient, whereas initiation of dapsone was associated with marked improvement of skin lesions. A group from Turkey in a later publication has reported a case of heterozygous protein C deﬁciency and LV [13]. They reported an 18-year-old man in whom pentoxyﬁllin in a combination with aspirin and dypiridamole resulted in improved skin lesions. Kavala et al. reported a case of 19-year-old male with LV associated with Factor V Leiden [14], which is believed to be the most common inherited procoagulant disorder [15]. Initiation of warfarin improved skin lesions in this patient. Biedermann et al. supported the association between Factor V Leiden and LV, by publishing another case report on this relationship [16]. Tissue plasminogen activator (TPA) is a key intrinsic regulator of clot lysis, and under normal conditions, it is bound to its biological inhibitor, known as plasminogen activator inhibitor (PAI) [17]. Mutations in PAI can increase its aﬃnity to TPA, thus decreasing the availability of TPA under circumstances when clot lysis is physiologically appropriate. Deng et al. reported a case report of PAI 4G/4G homozygosity and LV in a 33-year-old Caucasian female [18]. Warfarin and episodic administration of TPA led to an improvement. Agirbasli et al. found increased stability of PAI in patients with LV [19]. Antunes et al. reported a case of 25-year-old female with PAI 4G/4G homozygosity and prothrombin G20210A mutation [20]. Initiation of TPA has led to resolution of her condition. 3 A group from Germany has reported a case of 49-year- old female with high homocysteine levels and LV [21]. Com- mencement of vitamin B12, folic acid, and vitamin B6 sup- plementation led to a resolution of symptoms. A group from Lebanon has reported a case of 34-year-old female heterozy- gous for Factor V Leiden, prothrombin G20210A, and C677T methyltetrahydrofolate reductase (MTHFR) homozygosity [22]. C677T genotype of the MTHFR is known to be strongly associated with an increased cardiovascular risk, which is partly explained by prothrombotic eﬀects of homocysteine [22, 23]. Therapy with aspirin, vitamin B12, folic acid, and prednisone led to clinical improvement in their case. Several case reports have highlighted the association between G20210A genotype of the prothrombin gene and LV [4, 10, 22, 24–26]. However, in contrast to these reports, our patient had a moderately increased lupus anticoagulant (please see the Table 1), but aPTT, antiphospholipid antibodies, and ANA were negative. Important to mention that the recent study highlighted the possibility of a laboratory error in the measurement of lupus anticoagulant [27], and this might be relevant to our case. Another interesting ﬁnding in our patient is the absence of LV associated pain. To our knowledge, this is the ﬁrst case of painless ulcers in LV and this clinical presentation looks particularly intriguing. Our patient did not have diabetes mellitus, her motor function was not impaired and vibratory, light and pain/temperature sensation were preserved, which could potentially explain her clinical presentation. Noteworthy mentioning that dermal blood vessels are typically aﬀected and thus, it may explain why arterial Doppler ultrasound was negative, which has a limited sensitivity for small vessels. Several case reports highlight the beneﬁt of anticoagulation therapy, especially in patients with prothrombotic states. Several reports have highlighted the beneﬁcial role for LMWH in the therapy of LV [28, 29]. Our patient was on enoxaparin as well, which was discontinued after a stable therapeutic INR was achieved. In contrast to the vast majority of published case reports, our patient did not have any signiﬁcant history of thrombotic events and initially presented with LV in advanced age. This presentation may look somewhat contradictory, since it is clinically expected to have a history of prior thrombotic event at such age with a presumed procoagulant state. This may point toward the possibility that G20210 mutation did not inﬂuence the occurrence of LV in our patient. However, based on the prior literature, abnormal coagulation studies in our patient, diagnostic skin biopsy, and a good response to anticoagulant therapy, we think that it is likely that our patient had a procoagulant state-associated LV. Overall, this case report highlights a possibility of a painless LV presentation in a patient without signiﬁcant past thrombotic events. Therefore, it is essential to consider LV in the diﬀerential diagnosis even in a patient with painless ulcerative, atrophic, and/or nodular skin lesions over the shins and malleoli. Coagulation studies and autoimmune disease workup should be performed in patients with similar presentation and negative workup for chronic venous