External jugular veinous thrombosis: A rare presentation of Hansen's disease

Abstract

Veinous thrombosis is a rare manifestation of leprosy (Hansen's disease). In this case report, we bring to notice this extremely rare presentation and challenges faced during management. A 26-year-old young healthy individual presented with painless cord-like swellings on both sides of the neck for 1 month. This was evaluated to be external jugular vein (EJV) thrombosis on ultrasonography Doppler. Trying to evaluate the cause of the thrombosis, there was no specific etiology found. On further detailed evaluation, it was found that he had hypopigmented skin patches. This led to the diagnosis of borderline tuberculoid leprosy downgrading to borderline lepromatous in type 1 lepra reaction. He responded to rifampicin, clofazimine, and dapsone with progressive resolution of skin lesions. Simultaneous treatment with apixaban caused complete resolution of EJV thrombosis. Further evaluation revealed heterozygous methylenetetrahydrofolate reductase mutation and beta-2-glycoprotein immunoglobulin M positivity. An extensive literature review suggested that intimal damage caused by lepra bacilli can lead to veinous thrombosis in Hansen's disease. However, the interplay of genetic procoagulant predispositions in phenotypic presentation is yet to be determined.