Langerhans cell histiocytosis (LCH) is a disorder of unknown etiology characterized by proliferation of CD1a⁺ dendritic cells [1]. It occurs mainly in children. Adult LCH is a quite rare disease with an incidence of 1–2 cases per million [1]. LCH may present as a single organ system disease or multi-organ system disease. In the single organ system disease, skin, lung and bone involvement are common. Skin involvement is the most common type of presentation and lung involvement is the most severe form of the disease. Liver involvement is reported in 10.1–18% of the pediatric patients with LCH [2, 3]. Sclerosing cholangitis (SC) was found in 1.3–6% of children with LCH [2, 3]. In adults, the combination of SC and LCH is rarely reported. There are 15 cases in the literature which are reviewed in this paper.

A 65-year-old female was referred to our clinic with pruritus and elevated gamma-glutamyl transferase (GGT) level. Four years ago, she had had papular and pustular lesions and her skin biopsy revealed the diagnosis of LCH (Figs. 1, ​,2).2). Skin lesions disappeared with corticosteroid treatment but pruritus persisted. She also had diabetes and was using metformine and glimepiride. Physical examination revealed a palpable liver 4 cm below the costal margin. Laboratory analysis showed: ALT 45 U/l, AST 25 U/l, total bilirubin 0.76 mg/dl, conjugated bilirubin 0.21 mg/dl, alkaline phosphatase 177 U/l, GGT > 1543 U/l, albumin 2.1 g/dl, Ig G 1880 mg/dl (N: 700–1660), Ig M 137 mg/dl (N: 40–320), Ig A 353 mg/dl (N: 70–400), hemoglobin 13.2 g/dl, white blood cell count 10800/mm³, platelets 274000/mm³, protrombin time 10.2 s (107%), International normalized ratio (INR) 0.87. Serologic examination revealed: anti-HAV IgM (–), anti-HAV IgG (+), anti-HBc IgG (+), HBs Ag (–), Anti-HBs (+), anti-HCV (–), HCV-RNA (–), fluorescent antinuclear antibody (–), anti-smooth muscle antibody (–), anti-mitochondrial antibody (–). Radiological examination of the skull, vertebrae and long bones, chest X-ray and cranial CT scan were normal. Magnetic resonance cholangiopancreatography (MRCP) showed slight irregularities of intrahepatic ducts (Fig. 3). Liver biopsy revealed only steatosis with normal portal areas and biliary tracts. She was then lost to follow up. One year later, she presented with jaundice. She had pruritic skin lesions on her palms (Fig. 4). Laboratory tests revealed: ALT 20 U/l, AST 35 U/l, GGT 157 U/l, alkaline phosphatase 252 U/l, total bilirubin 25.3 mg/dl, conjugated bilirubin > 15 mg/dl, INR 1.39, albumin 2.17 g/dl, hemoglobin 12.1 g/l, white blood cell count 15,200/mm³. MRCP showed multifocal strictures and irregularity of the intrahepatic bile ducts. Endoscopic Retrograde Cholangiopancreatography revealed narrowing of the intrahepatic bile ducts and common bile duct, a plastic stent of 10 F diameter and 12 cm of length was inserted into common bile duct which had been obliterated. A second liver biopsy was performed which showed bile stasis, bile duct damage, ductular proliferation, mixed inflammation and mild fibrosis, but there was no Langerhans cell histiocyte infiltration (Figs. 5, ​,6).6). Immunohistochemical stain with CD1a which is a marker of Langerhans type dendritic cells was also negative. Histopathological findings were considered compatible with sclerosing cholangitis, however they were not definitely diagnostic. We discharged the patient with 1 g per day of ursodeoxycholic acid therapy. Eight months later, during her last control, she was still icteric and complaining of pruritus. Her laboratory tests revealed: ALT 69 U/l, AST 109 U/l, GGT 573 U/l, alkaline phosphatase 330 U/l, total bilirubin 13 mg/dl, conjugated bilirubin 10 mg/dl, INR 1, albumin 2.1 g/dl. She was still using ursodeoxycolic acid in the same dose.