Severe secondary sclerosing cholangitis as manifestation of a very rare underlying disease

Langerhans cell histiocytosis (LCH) is a very rare cause of secondary sclerosing cholangitis. We report the case of a 42-year-old male patient with sclerosing cholangitis and histological evidence of LCH from a bile duct biopsy. Due to rapid disease progression and exhaustion of conservative therape...

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Bibliographic Details
Published in:Der Internist (Berlin) 2021-12, Vol.62 (12), p.1349
Main Authors: Zecher, Britta Franziska, Zenouzi, Roman, Lang, Melanie, Karagiannis, Panagiotis, Clauditz, Till, Fischer, Lutz, Sterneck, Martina, Schramm, Christoph, Lohse, Ansgar W, Sebode, Marcial
Format: Article
Language:ger
Subjects:
Adult
Biopsy
Cholangitis, Sclerosing - diagnosis
Cholangitis, Sclerosing - therapy
Histiocytosis, Langerhans-Cell
Humans
Liver Transplantation
Male
Rare Diseases
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Summary:Langerhans cell histiocytosis (LCH) is a very rare cause of secondary sclerosing cholangitis. We report the case of a 42-year-old male patient with sclerosing cholangitis and histological evidence of LCH from a bile duct biopsy. Due to rapid disease progression and exhaustion of conservative therapeutic approaches the patient received a liver transplantation. Nearly 2 years after transplantation the patient has a good graft function and no signs of recurrence of the underlying LCH.
ISSN:1432-1289
DOI:10.1007/s00108-021-01148-0