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Journal of the American Academy of Dermatology
Volume 63, n° 2
pages 284-291 (août 2010)
Doi : 10.1016/j.jaad.2009.08.013

Combined Erdheim-Chester disease and Langerhans cell histiocytosis of skin are both monoclonal: A rare case with human androgen-receptor gene analysis

Jen-Wei Tsai, MD a, Jen-Hui Tsou, MS c, Liang-Yi Hung, PhD c, Hung-Bo Wu, MD b, Kung-Chao Chang, MD d,
a Department of Anatomic Pathology, E-Da Hospital, Kaohsiung County, Taiwan 
b Division of Hematology-Oncology, Department of Internal Medicine, E-Da Hospital, Kaohsiung County, Taiwan 
c Institute of Biosignal Transduction, College of Bioscience and Biotechnology, Tainan, Taiwan 
d Department of Pathology, College of Medicine, National Cheng Kung University, Tainan, Taiwan 

Reprint requests: Kung-Chao Chang, MD, Department of Pathology, National Cheng Kung University Hospital, 138 Sheng-Li Road, Tainan 704, Taiwan.

Erdheim-Chester disease (ECD) is a rare xanthogranulomatous histiocytic disorder. Langerhans cell histiocytosis (LCH) is a proliferative disorder of histiocytes with a phenotype similar to dendritic Langerhans cells. Both are derived from myeloid stem cells in the bone marrow and, thus, can overlap.


We report a rare case of hybrid LCH and ECD of the skin with systemic ECD.


Pathologic examinations and human androgen-receptor gene assay were used to study this case.


A 34-year-old woman presented with recurrent ulcerative skin lesions on both thighs associated with polydipsia and polyuria since childhood. Radiography revealed osteosclerosis of bilateral distal tibias and soft tissue masses of bilateral chest walls and ankles. Pathologically, the chest wall lesions showed dense aggregates of lipid-laden histiocytes, which were CD68+/CD163+/S100/CD1a. Combined with the clinical and radiographic findings, this xanthogranulomatous infiltrate was consistent with ECD. However, thigh skin showed discrete foci of a xanthogranulomatous infiltrate and S100+/CD1a+ Langerhans cells with eosinophils. In addition, Birbeck granules were found. Dissected tissues from both ECD and LCH were monoclonal, supporting their neoplastic nature.


Single case report is a limitation.


ECD and LCH may have a close association with divergent differentiation from the same stem cells under different microenvironmental conditions.

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Key words : Erdheim-Chester disease, human androgen-receptor gene analysis, hybrid lesion, Langerhans cell histiocytosis, monoclonal, skin

Abbreviations used : ECD, HUMARA, LCH

 Funding sources: None.
 Conflicts of interest: None declared.

© 2009  American Academy of Dermatology, Inc.@@#104156@@