Abstract
Mondor’s disease is a rare condition and usually treated with low-molecular weight heparin and non-steroidal anti-inflammatory drugs. Because of paucity of cases and for the usually spontaneous resolution, there is not a standard treatment strategy and the use of oral anticoagulation in controversial. We reported the efficacy of direct oral anticoagulants in the recurrent Mondor’s disease refractory to standard therapy.
Report
Mondor’s disease (MD) is a rare condition characterized by the onset of subcutaneous thrombophlebitis. The sites affected are usually the anterolateral thoracoabdominal wall and dorsolateral vein of penis. 1 In some cases, MD may be secondary to other diseases such as malignancies or to a thrombophilic state.2,3 The diagnosis is based on clinical signs, while Doppler ultrasound is an instrument of choice. Primary MD usually resolves in four to eight weeks without any intervention. In cases of painful MD, first-line therapy consists of non-steroidal anti-inflammatory drugs (NSAIDs) and/or low-molecular weight heparin (LMWH). 4 There is not a standard treatment strategy for MD because of its rarity and the use of oral anticoagulation is controversial. We present our experience about the treatment with direct oral anticoagulants (DOACs) in MD occurred as a manifestation of recurrent venous thromboembolism (VTE) and refractory to conventional therapies. The first case is about a 46-year-old man already on anticoagulant therapy with vitamin K antagonists (VKA) for VTE since 1996. On May 2018, he presented a deep venous thrombosis (DVT) of both lower limbs associated with penile MD (Figure 1). VKA treatment was temporarily replaced with fondaparinux 7.5 mg daily. After a month, DOAC apixaban was started because of thrombosis and MD pain persistence. This anticoagulant therapy was effective in resolving patient’s thromboses and relieving MD symptoms. The second case is a 46-year-old woman, on VKA therapy for recurrent VTE. She was admitted to our site on October 2018 because of the onset of a painful cord on the right chest wall. Ultrasound documented MD of thoracoepigastric vein. VKA therapy was firstly replaced with LMWH therapeutic dose. After two months, given the persistence of MD and the onset of a popliteal DVT, DOAC therapy with apixaban was started. MD clinical signs rapidly improved during the first month of DOAC as well as thrombosis of the lower limbs. The third case regards a 49-year-old man with a history of frequent thrombophlebitis, all treated with on-demand LMWH until resolution. His clinical course was complicated by two episodes of penal MD on September 2010 and September 2012, requiring association treatment with LMWH and NSAIDs. Thrombophilic screening documented heterozygosity of Factor V Leiden mutation, while JAK2 V617F and BCR-ABL mutations were negative. On March 2016, the patient presented a third MD episode associated with left femoral DVT. Despite LMWH therapy, after three months, MD pain symptoms did not relief and thrombosis persisted. DOAC therapy with rivaroxaban was started and the complete resolution of both MD and DVT was observed after a month of treatment. The last case is a 29-year-old patient with a protein C deficiency, on anticoagulant therapy with VKA because of recurrent VTE since 2017. On October 2018, he presented a penile MD. Despite therapeutic LMWH, MD got worse. Therefore, we decided to start DOAC therapy with rivaroxaban. After a month of treatment, the pain disappeared as well as the induration on penis dorsal surface.
Patient 1 magnetic resonance imaging at diagnosis showing T2 hyperintense signal due to thrombosis of the dorsal vein of the penis.
Discussion
MD is rare and consists of an occluded subcutaneous vessel with an acute onset. Two forms of MD exist: a primary MD usually idiopathic and a secondary MD due to an underlying disease, such as hypercoagulative state; autoimmune disorders with vasculitis; malignancies. 5 Because of paucity of cases and for the frequent spontaneous resolution, there is not a standard treatment strategy and the use of oral anticoagulation in controversial. In fact, LMWH and NSAIDs are usually the standard approach that can resolve this type of thrombotic event. In our patients, DOACs proved to be effective in MD, although they were started after the failure of the conventional anticoagulant treatments in resolving thromboses and preventing recurrent VTE. In conclusion, our experience suggests that DOACs may be an alternative treatment in this rare thrombotic disease, in case of failure of NSAIDs and traditional anticoagulant therapy.
Footnotes
Declaration of Conflicting Interests
The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding
The author(s) received no financial support for the research, authorship, and/or publication of this article.
Ethical approval
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Contributorship
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Acknowledgements
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