• Langerhans cell histiocytosis
• cholestasis
• liver cirrhosis
• liver transplantation
• sclerosing cholangitis

• abdominal pain
• biliary dyskinesia
• histiocytosis, langerhans-cell
• liver neoplasms

• Male
• Humans
• Middle Aged
• Proto-Oncogene Proteins B-raf/genetics
• Mutation
• Histiocytosis, Langerhans-Cell/diagnosis
• Liver
• Abdominal Pain

Hepatic Langerhans cell histiocytosis (LCH) is characterized by proliferation and accumulation of Langerhans cells in the liver, causing liver dysfunction or forming a mass lesion. The liver can be involved in isolation, or be affected along with other organs. A common clinical hepatic presentation is cholestasis with pruritis, fatigue and direct hyperbilirubinemia. In late stages, there may be hypoalbuminemia. Liver biopsy may be required for the diagnosis of hepatic LCH. Histologic finding may be diverse, including lobular Langerhans cell infiltrate with mixed inflammatory background, primary biliary cholangitis-like pattern, sclerosing cholangitis-like pattern, and even cirrhosis at later stages. Because of its non-specific injury patterns with broad differential diagnosis, establishing a diagnosis of hepatic LCH can be challenging. Hepatic LCH can easily be missed unless this diagnosis is considered at the time of biopsy interpretation. A definitive diagnosis relies on positive staining with CD1a and S100 antigen. Liver involvement is a high risk feature in LCH. The overall prognosis of hepatic LCH is poor. Treating at an early stage may improve the outcome. Systemic chemotherapy is the mainstay of treatment and liver transplantation may be offered. New molecular markers involved in pathogenesis of LCH are being explored with a potential for targeted therapy. However, further studies are needed to improve outcome.

• Langerhans cell
• Liver
• Cholangitis
• CD1a

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 within the florid duct lesion were positive for CD1a and S-100. Concurrently, a small focus of LCH was noted in his gastric biopsy performed for gastritis symptoms. Hepatic LCH may mimic AMA-negative PBC histologically and clinically and may present as a harbinger of multisystem LCH. While rendering the diagnosis would be challenging without prior history of LCH and with focal involvement, awareness of such presentation and communication with clinical colleagues may be helpful.

• cholangitis
• histiocytosis
• langerhans
• liver

Langerhans cell histiocytosis (LCH) is a rare childhood hematopoietic disease, and hence, there are few reports summarizing the course leading to the diagnosis. We described the initial symptoms and the clinical course of LCH. We carried out a retrospective review of charts from a single medical center, and 21 patients with the diagnosis of LCH were enrolled. The initial symptoms of 16 cases were caused by bone lesions; of these cases, there were 8 instances of soft tissue swelling as the initial symptom (38%) and 8 instances of bone pain without swelling (38%). Among the cases of bone lesion, 4 of 6 cases of skull lesion were painless while all vertebral body lesions and long bone lesions were accompanied by pain. LCH bone lesions caused various symptoms depending on the site of the lesion and this makes the diagnosis difficult. A detailed physical examination and imaging studies are recommended for early diagnosis.

• Langerhans cell histiocytosis
• bone lesion
• initial symptom

• Cholestasis
• Lymphadenopathy
• Porta hepatis