Hepatic Langerhans Cell Histiocytosis (LCH) Presenting as a Harbinger of Multisystem LCH

Curēus
Hua LiHwajeong Lee

Abstract

Langerhans cell histiocytosis (LCH) is a rare systemic disorder characterized by an infiltration of CD1a+/langerin+ histiocytes, commonly involving bone, skin, and lymph nodes in children. Hepatic involvement is rarely observed in multisystem LCH. We describe an exceptional case of hepatic LCH in an adult preceding the diagnosis of multisystem LCH, mimicking anti-mitochondrial antibody (AMA)-negative primary biliary cholangitis (PBC). A 65-year-old man presented with intermittent pruritus, weakness, dyspnea, fever, and chills that have been progressive for four years. Physical examination was unremarkable. Laboratory work revealed cholestatic biochemistry profile. Liver biopsy showed portal non-necrotizing granuloma encasing a damaged duct (florid duct lesion), and multifocal lobular Kupffer cell clusters, suggestive of PBC. Tests for autoimmune diseases including AMA were negative. Endoscopic retrograde cholangiopancreatography (ERCP) was negative for biliary obstruction. One month after the liver biopsy, he developed flaky, red, and burning rash on the right scalp, forehead, and epigastric skin. A skin biopsy at an outside institution revealed LCH. Subsequent re-examination of the liver biopsy showed that the histiocytes with...Continue Reading

References

May 10, 2011·Clinics and Research in Hepatology and Gastroenterology·Meya AbdallahJean Cabane
Sep 13, 2014·Journal of Digestive Diseases·Danying ZhangXizhong Shen
Aug 30, 2018·The New England Journal of Medicine·Carl E AllenKenneth L McClain
Feb 28, 2020·Blood·Carlos Rodriguez-Galindo, Carl E Allen

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Citations

Jun 17, 2021·World Journal of Clinical Oncology·Zhiyan FuHwajeong Lee

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Methods Mentioned

BETA
biopsy

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