Case report: Development of vanishing bile duct syndrome in Stevens-Johnson syndrome complicated by hemophagocytic lymphohistiocytosis
BackgroundVanishing 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 lymphohisti...
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Frontiers Media S.A.
2022-10-01
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| Series: | Frontiers in Medicine |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fmed.2022.975754/full |
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| author | Wan-Chen Lin Wan-Chen Lin Tyng-Shiuan Hsieh Tyng-Shiuan Hsieh Chia-Yu Chu Chia-Yu Chu |
| author_facet | Wan-Chen Lin Wan-Chen Lin Tyng-Shiuan Hsieh Tyng-Shiuan Hsieh Chia-Yu Chu Chia-Yu Chu |
| author_sort | Wan-Chen Lin |
| collection | DOAJ |
| description | BackgroundVanishing 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 presentationWe 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 clinical lab data clues, as well as repeated liver biopsies showing recovering ductopenia, the patient developed hypovolemic shock combined with septic shock episodes and died on day 236.ConclusionTo our knowledge, this is the fifteenth report of vanishing bile duct syndrome associated with Stevens-Johnson disease or toxic epidermal necrolysis. Mortality rate remains high without treatment guidelines established due to the rarity and heterogenicity of the population. Further studies are needed to identify possible risk factors, prognostic indicators, and the standard of care for vanishing bile duct syndrome associated with Stevens-Johnson disease or toxic epidermal necrolysis. |
| first_indexed | 2024-04-11T08:42:20Z |
| format | Article |
| id | doaj.art-ba7beb867c4b4de6a54a7aa17bdf507b |
| institution | Directory Open Access Journal |
| issn | 2296-858X |
| language | English |
| last_indexed | 2024-04-11T08:42:20Z |
| publishDate | 2022-10-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Medicine |
| spelling | doaj.art-ba7beb867c4b4de6a54a7aa17bdf507b2022-12-22T04:34:11ZengFrontiers Media S.A.Frontiers in Medicine2296-858X2022-10-01910.3389/fmed.2022.975754975754Case report: Development of vanishing bile duct syndrome in Stevens-Johnson syndrome complicated by hemophagocytic lymphohistiocytosisWan-Chen Lin0Wan-Chen Lin1Tyng-Shiuan Hsieh2Tyng-Shiuan Hsieh3Chia-Yu Chu4Chia-Yu Chu5Department of Dermatology, National Taiwan University Hospital, Taipei, TaiwanDepartment of Dermatology, National Taiwan University College of Medicine, Taipei, TaiwanDepartment of Dermatology, National Taiwan University Hospital, Taipei, TaiwanDepartment of Dermatology, National Taiwan University College of Medicine, Taipei, TaiwanDepartment of Dermatology, National Taiwan University Hospital, Taipei, TaiwanDepartment of Dermatology, National Taiwan University College of Medicine, Taipei, TaiwanBackgroundVanishing 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 presentationWe 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 clinical lab data clues, as well as repeated liver biopsies showing recovering ductopenia, the patient developed hypovolemic shock combined with septic shock episodes and died on day 236.ConclusionTo our knowledge, this is the fifteenth report of vanishing bile duct syndrome associated with Stevens-Johnson disease or toxic epidermal necrolysis. Mortality rate remains high without treatment guidelines established due to the rarity and heterogenicity of the population. Further studies are needed to identify possible risk factors, prognostic indicators, and the standard of care for vanishing bile duct syndrome associated with Stevens-Johnson disease or toxic epidermal necrolysis.https://www.frontiersin.org/articles/10.3389/fmed.2022.975754/fullallergynon-steroidal anti-inflammatory drugsdrug-induced liver injuryvanishing bile duct syndromeStevens-Johnson syndromehemophagocytic lymphohistiocytosis |
| spellingShingle | Wan-Chen Lin Wan-Chen Lin Tyng-Shiuan Hsieh Tyng-Shiuan Hsieh Chia-Yu Chu Chia-Yu Chu Case report: Development of vanishing bile duct syndrome in Stevens-Johnson syndrome complicated by hemophagocytic lymphohistiocytosis Frontiers in Medicine allergy non-steroidal anti-inflammatory drugs drug-induced liver injury vanishing bile duct syndrome Stevens-Johnson syndrome hemophagocytic lymphohistiocytosis |
| title | Case report: Development of vanishing bile duct syndrome in Stevens-Johnson syndrome complicated by hemophagocytic lymphohistiocytosis |
| title_full | Case report: Development of vanishing bile duct syndrome in Stevens-Johnson syndrome complicated by hemophagocytic lymphohistiocytosis |
| title_fullStr | Case report: Development of vanishing bile duct syndrome in Stevens-Johnson syndrome complicated by hemophagocytic lymphohistiocytosis |
| title_full_unstemmed | Case report: Development of vanishing bile duct syndrome in Stevens-Johnson syndrome complicated by hemophagocytic lymphohistiocytosis |
| title_short | Case report: Development of vanishing bile duct syndrome in Stevens-Johnson syndrome complicated by hemophagocytic lymphohistiocytosis |
| title_sort | case report development of vanishing bile duct syndrome in stevens johnson syndrome complicated by hemophagocytic lymphohistiocytosis |
| topic | allergy non-steroidal anti-inflammatory drugs drug-induced liver injury vanishing bile duct syndrome Stevens-Johnson syndrome hemophagocytic lymphohistiocytosis |
| url | https://www.frontiersin.org/articles/10.3389/fmed.2022.975754/full |
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