A RARE MULTISYSTEM LANGERHANS CELL HISTIOCYTOSIS PRESENTED WITH SCLEROSING CHOLANGITIS: CASE REPORT AND LITERATURE REVIEW

JIN-DONG FU¹, HUI WANG^1*, LING WANG¹, YAO LI2, YUGUO ZHANG

¹MD People’s Hospital of Rizhao, 126 Taian RoadDonggang District，Rizhao,276826, Shandong Province, P R China - ²The Third Hospital of Hebei Medical University, Ziqiang Road, Shijiazhuang City 050051, Hebei Province, P R China

Introduction: Langerhans cell histiocytosis is a rare disease with unknown etiology. It occurs more in children and also in adults and involves one or more organs such as skin, bone, lung, hypothalamus, posterior pituitary gland, lymph nodes and other tis- sues. But liver involvement is rarely reported and pathological manifestation of sclerosing chlongitis is rare.

Metholds: We reported a case of langerhans cell histocytosis with multisystem involvement including skin, lungs, thyroid gland, rare liver, nails and possible spleen in a young adult man.

Results: Though he developed cholestasis and came to liver disease center firstly, he was diagnosed by infiltration of Langerhans cell in the skin and thyroid gland biopsy, with pathological manifestation of sclerosing cholongitis in liver biopsy.

Conclusion: It is a rare case with multi-organ involved.