Pemphigus vulgaris with thromboembolism: A case report and literature review

Pemphigus vulgaris (PV) is a rare and potentially fatal autoimmune blistering disease. PV can be associated with an increased risk of thrombosis, with studies demonstrating a clear association between pemphigoid disease and an increased risk of venous thromboembolism (VTE). The etiology of VTE in PV is unknown but is likely multifactorial. The presence of high titers of autoantibodies against desmogleins and treatment with high-dose corticosteroids in acute pemphigus flares are thought to be associated with an increased risk of VTE. We describe the case of a man with severe, intractable mucocutaneous PV covering more 60% of his total body surface area, on a high-dose corticosteroid regimen, who developed extensive deep vein thrombosis (DVT) and bilateral pulmonary embolisms (PEs) despite adequate systemic DVT/PE prophylaxis, and who subsequently required therapeutic anticoagulation.