Case report: Development of vanishing bile duct syndrome in Stevens-Johnson syndrome complicated by hemophagocytic lymphohistiocytosis.

ABSTRACT:

Background

Vanishing bile duct syndrome is a rare drug-induced disease characterized by cholestasis and ensuing ductopenia. Dermatological manifestations of drug hypersensitivity such as Stevens-Johnson syndrome and toxic epidermal necrolysis may also present in such cases. Hemophagocytic lymphohistiocytosis is a hyperimmune response caused by unchecked stimulation of macrophages, natural killer cells, and cytotoxic T lymphocytes.

Case presentation

We report a severe case who presented with concurrent Stevens-Johnson syndrome and vanishing bile duct syndrome complicated by hemophagocytic lymphohistiocytosis after the ingestion of non-steroidal anti-inflammatory drugs. Despite the fact that improvements in vanishing bile duct syndrome can be assumed when combining the clinica

SUBMITTER: Lin WC 

PROVIDER: S-EPMC9637682 | biostudies-literature | 2022

REPOSITORIES: biostudies-literature