Multisystem Langerhans cell histiocytosis with hypothyroidism and sclerosing cholangitis: a case report and review of the literature.
- 1Department of Infectious Diseases, Shandong Public Health Clinical Center, Shandong University, Jinan, 250013, Shandong, China.
- 2Department of Liver Diseases, Shandong Public Health Clinical Center, Shandong University, No.2999 Gangxing West Road, Licheng District, Jinan, 250013, Shandong, China.
- 3Department of Liver Diseases, Shandong Public Health Clinical Center, Shandong University, No.2999 Gangxing West Road, Licheng District, Jinan, 250013, Shandong, China. [email protected].
Abstract
Background
Langerhans cell histiocytosis (LCH) is a rare disease caused by a disorder of dendritic cells. LCH mainly occurs in children and the damage of tissue is caused by proliferative dendritic cells which also provides important evidence for the diagnosis of this disease. In most LCH patients, only a single organ in the body is affected; however, multisystem involvement may occur, commonly affecting the bone marrow, liver, lungs, and central nervous system. In this report, we describe a case of multisystem LCH involving the skin, lungs, thyroid gland, liver, nails, and spleen.Case introduction
A 20-year-old Han Chinese man was admitted into the liver disease department with complaints of anorexia, fatigue, and jaundice. Physical examination revealed extensive cutaneous lesions, nail lesions, macular sclera, and bilateral goiter. Lymphocytic thyroiditis was diagnosed based on fine-needle aspiration biopsy of the thyroid gland and corroborating clinical features-including the characteristic skin rash. Liver biopsy confirmed sclerosing cholangitis (SC). Response to treatment with Etoposide (VP-16), Prednisone acetate tablets, cyclophosphamide, and vinblastine was excellent. However, the erythrocyte sedimentation rate (ESR) was initially elevated above the normal reference range. With disease remission, the corresponding ESR levels decreased.Conclusions
LCH in this case involved not only the skin and thyroid, but also the liver, lungs, nails and possibly the spleen. Currently there are no standard treatment options and treatment needs to be individualized. It is rare that LCH with sclerosing cholangitis is the first presentation, and we should be vigilant to whether other organs are involved. An elevated ESR may be a valuable indicator of disease activity in patients with Langerhans cell histiocytosis.
Related
- Extensive vulvar and perianal langerhans cell histiocytosis with clinical improvement during low-dose thalidomide and topical therapy: a case report
- Adult-onset Langerhans cell histiocytosis in an elderly female with cutaneous and nodal involvement: A case report
- Langerhans Cell Histiocytosis of the Vulva: A Pediatric Case.
- No detectable infectious agents in Langerhans cell histiocytosis with lung involvement
- Fine‐Needle Aspirate Cytology of Feline Pulmonary Langerhans Cell Histiocytosis in Two Cats
BibTeX
@article{yu2026multisystem,
title = {{Multisystem Langerhans cell histiocytosis with hypothyroidism and sclerosing cholangitis: a case report and review of the literature.}},
author = {Jinhong Yu and Xiaoyan Liu and Xia Yu and Qiang Li and Wenjun Du},
journal = {Journal of medical case reports},
year = {2026},
month = aug,
doi = {10.1186/s13256-026-06454-5},
url = {https://europepmc.org/article/MED/42968175},
note = {CC BY-NC-ND},
}
RIS
TY - JOUR
TI - Multisystem Langerhans cell histiocytosis with hypothyroidism and sclerosing cholangitis: a case report and review of the literature.
AU - Jinhong Yu
AU - Xiaoyan Liu
AU - Xia Yu
AU - Qiang Li
AU - Wenjun Du
PY - 2026
DA - 2026/08/05
JO - Journal of medical case reports
DO - 10.1186/s13256-026-06454-5
UR - https://europepmc.org/article/MED/42968175
AB - Background Langerhans cell histiocytosis (LCH) is a rare disease caused by a disorder of dendritic cells. LCH mainly occurs in children and the damage of tissue is caused by proliferative dendritic cells which also provides important evidence for the diagnosis of this disease. In most LCH patients, only a single organ in the body is affected; however, multisystem involvement may occur, commonly affecting the bone marrow, liver, lungs, and central nervous system. In this report, we describe a case of multisystem LCH involving the skin, lungs, thyroid gland, liver, nails, and spleen.Case introduction A 20-year-old Han Chinese man was admitted into the liver disease department with complaints of anorexia, fatigue, and jaundice. Physical examination revealed extensive cutaneous lesions, nail lesions, macular sclera, and bilateral goiter. Lymphocytic thyroiditis was diagnosed based on fine-needle aspiration biopsy of the thyroid gland and corroborating clinical features-including the characteristic skin rash. Liver biopsy confirmed sclerosing cholangitis (SC). Response to treatment with Etoposide (VP-16), Prednisone acetate tablets, cyclophosphamide, and vinblastine was excellent. However, the erythrocyte sedimentation rate (ESR) was initially elevated above the normal reference range. With disease remission, the corresponding ESR levels decreased.Conclusions LCH in this case involved not only the skin and thyroid, but also the liver, lungs, nails and possibly the spleen. Currently there are no standard treatment options and treatment needs to be individualized. It is rare that LCH with sclerosing cholangitis is the first presentation, and we should be vigilant to whether other organs are involved. An elevated ESR may be a valuable indicator of disease activity in patients with Langerhans cell histiocytosis.
SN - 1752-1947
ER -