Recurrent venous thrombosis in an adequately anticoagulated patient with pemphigus vulgaris

Abstract

BackgroundSeveral autoimmune skin disorders are characterised by an increased risk of thrombosis, with bollous pemphigoid carrying a higher risk than pemphigus vulgaris (PV). We describe the case of a middle aged gentleman who developed recurrent venous thromboembolism despite adequate oral anticoagulation during very active PV that required escalation of treatment to bring the disease under control.Case presentationIn May 2014 a 49 year gentleman was admitted for widespread mucocutaneous blistering diagnosed as PV by histology and immunofluorescence. After 6 weeks of treatment with systemic steroids and azathioprine the patient developed pulmonary emboli and started oral anticoagulation with warfarin. In late September, the patient re-presented with a severe flare of PV and a recurrent deep vein thrombosis despite oral anticoagulation within therapeutic range. Warfarin was changed to subcutaneous low molecular heparin in therapeutic dose while treatment for pemphigus was escalated: first azathioprine was switched to mycophenolate mofetil and the steroids dose increased; then due to poor response, intravenous immunoglobulins were given for three courses and finally he received four infusions of Rituximab that induced sustained remission. In April 2015 the dose of mycophenolate was decreased but anticoagulation was continued until the beginning of July 2015 to ensure that decreasing immune suppression did not allow the emergence of another flare with attendant thrombotic risk.ConclusionThe case highlights the risk of thrombosis and re-thrombosis in aggressive PV and demands further clinical research in this area to assess the need for thromboprophylaxis in aggressive bollous skin disease.

A Case of Duodenal Resection and Duodenojejunostomy for Multiple Small Bowel Infarction in Patient With Inherited Thrombophilia and Vitamin K Antagonist Induced Critical Hypocoagulation

“…Thrombosis despite anticoagulation could be caused by some diseases: pemphigus vulgaris [ 14 ], cancer [ 15 ], antiphospholipid antibody syndrome, obesity, a neurologic disease with extremity paresis, pregnancy, drug administration (e.g., oral contraceptives), etc. or inherited disorders: antithrombin deficiency, protein C deficiency, protein S deficiency, LFV.…”

Successful Thrombolysis for High-Risk Pulmonary Embolism in a Patient With Extensive Pemphigus Vulgaris

“…11 Considering other potential risk factors such as immobilization, or comorbidities, these patients may warrant assessment of the risk benefit ratio of thromboprophylaxis. 3,12 However, the bleeding risk because of the extensive cutaneous lesions and the association of corticotherapy may lead to underutilization of thromboprophylaxis in hospitalized patients. 4,7…”

Atypical Presentation of Pemphigus Vulgaris with Co-Existing Carcinoma Lung and Acute Onset Internal Jugular Vein Thrombosis

“…[ 1 ] In PV with a prolonged disease course and treatment, uncommon complications like vascular thromboembolism have been reported. [ 2 3 ] We present a case of treatment-naïve patient with atypical PV who developed sudden-onset life-threatening right internal jugular vein (IJV) thrombosis and was detected to have co-existing lung carcinoma.…”

“…[ 3 ] Unlike all previous reports, where vascular thrombosis occurred in pemphigus patients after prolonged treatment, this patient developed thrombosis before any treatment and within a few weeks of developing lesions. [ 2 3 ]…”