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

Journal of the American Academy of Dermatology
Jen-Wei TsaiKung-Chao Chang

Abstract

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 natu...Continue Reading

References

Aug 1, 1975·The Journal of Pediatrics·M E Lahey
Jul 21, 1994·The New England Journal of Medicine·C L WillmanD G Gilliland
Apr 1, 1996·Journal of the American Academy of Dermatology·D H ChiJ K Koh
Jun 18, 2002·Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc·Samer Al-QuranLisa Rimsza
Jun 19, 2002·Journal of Cranio-maxillo-facial Surgery : Official Publication of the European Association for Cranio-Maxillo-Facial Surgery·Arnd BartnickRainer Schmelzle
Apr 30, 2003·Advances in Anatomic Pathology·Michele BiscegliaThomas V Colby
Sep 1, 2004·Virchows Archiv : an International Journal of Pathology·V P AndradeW S L Goulart
Mar 22, 2006·European Journal of Haematology·Marcus Stockschlaeder, Christoph Sucker
Jan 27, 2007·The American Journal of Surgical Pathology·Eneida F VencioAndre M Oliveira
May 12, 2007·Skeletal Radiology·Paul S FurmanczykBrian P Rubin
Jan 12, 2008·Virchows Archiv : an International Journal of Pathology·Brendan Craig DicksonBharati Bapat
Jul 22, 2008·Journal of the European Academy of Dermatology and Venereology : JEADV·R KerzlM Mempel

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Citations

Nov 18, 2011·Current Opinion in Rheumatology·Julien HarocheZahir Amoura
Sep 10, 2013·Orphanet Journal of Rare Diseases·Roei D MazorYehuda Shoenfeld
Feb 18, 2014·Current Rheumatology Reports·Julien HarocheZahir Amoura
Nov 10, 2013·Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc·Kung-Chao ChangL Jeffrey Medeiros
Jul 22, 2015·Expert Review of Clinical Immunology·Julien HarocheZahir Amoura
Apr 20, 2013·Rheumatic Diseases Clinics of North America·Julien HarocheZahir Amoura
Nov 29, 2014·British Journal of Haematology·Marie-Luise BerresCarl E Allen
Jun 1, 2014·La Revue de médecine interne·J HarocheZ Amoura
Jan 1, 2011·Case Reports in Hematology·Peter G PavlidakeyHoward J Meyerson
Jun 5, 2014·Blood·Baptiste HervierUNKNOWN French Histiocytoses Study Group
Mar 2, 2013·Blood·Mark L Heaney
May 29, 2016·La Presse médicale·Julien HarocheZahir Amoura
Nov 19, 2015·Case Reports in Pathology·Shahzaib NabiRana Awdish
Oct 4, 2012·Chest·Marie ConradSebastien Gibot
Oct 14, 2017·BMJ Case Reports·Cristian Ricardo CalandraNaomi Arakaki
Aug 16, 2016·The American Journal of Surgical Pathology·Kung-Chao ChangL Jeffrey Medeiros
Mar 13, 2016·Journal of Cutaneous Pathology·José M MartínCarlos Monteagudo
Sep 25, 2020·Leukemia & Lymphoma·Arturo Bonometti, UNKNOWN for Associazione Italiana Ricerca Istiocitosi AIRI ONLUS
Jul 9, 2020·Journal of Clinical Imaging Science·Hema MeraiUday Mandalia
May 17, 2021·Neuro-oncology·Fleur Cohen AubartEli L Diamond

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