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

摘要

To the Editor: INTRODUCTION: Pemphigus vulgaris is a rare autoimmune disease caused by IgG autoantibodies directed against desmo-glein 3 or 1, transmembrane desmosomal glycopro-teins, causing acantholysis and intraepidermal blister formation. Mainstay of treatment consists of glucocor-ticoids and immunosuppressive therapy. Morbi-mortality is very high, because of the complications of the disease or of the treatment. Pemphigus patients are at an increased risk of venous thromboembolism (VTE), partly because of the immune-mediated substrate. This is especially prevalent in the first year after diagnosis, when the highest incidence of aggressive immunosuppressive treatment and its complications are also recorded. Oral glucocorticoids may increase the patients' risk of pulmonary embolism, mainly in the first month of treatment, notably in high-dose regimens. It is however uncertain whether the higher thromboembolism risk is caused by the corticosteroids, the underlying disease, or both.