Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Langerhans cell histiocytosis (LCH) of the alimentary tract is a rare condition, and LCH of the stomach especially so. We report a histologically and endoscopically unusual case of gastric LCH, the diagnosis of which was based on gastric mucosal biopsy specimens from an adolescent with multi-organ LCH and striking gastric polyposis. Microscopically, the gastric mucosa was expanded by discrete granulomatous microaggregates of Langerhans cells, resulting in a close resemblance to other, more common nonnecrotizing granulomatous gastritides. The diagnosis of LCH was confirmed immunohistochemically and ultrastructurally. To our knowledge, this is the first report of gastric LCH with a granulomatous histologic pattern, and the third to cause diffuse gastric polyposis. Langerhans cell histiocytosis should be included in the histologic differential diagnosis of granulomatous gastritis, as well as in the endoscopic differential diagnosis of gastric polyposis.
 ...
PMID:Langerhans cell histiocytosis of the stomach. A cause of granulomatous gastritis and gastric polyposis. 797 22

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.
 ...
PMID:Hepatic Langerhans Cell Histiocytosis (LCH) Presenting as a Harbinger of Multisystem LCH. 3267 32