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Langerhans Cell Histiocytosis

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Deadly Dermatologic Diseases

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Abstract

Langerhans cell histiocytosis (LCH) refers to a collection of syndromes, characterized by infiltration of various tissues by Langerhans cells (LC). Langerhans cells are dendritic antigen-presenting cells that normally reside within squamous epithelium, peri-epithelial connective tissue, and lymphatics and in areas of lymph node. Studies to date suggest that LCH is a heterogeneous disease with an unclear etiology. A clonal expansion of Langerhans cells has been demonstrated in many cases.

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References

  1. Farber S. The nature of “solitary or eosinophilic granuloma” of bone. Am J Pathol. 1941;17:625–9.

    Google Scholar 

  2. Lichtenstein L. Histiocytosis X, integration of eosinophilic granuloma of bone, Letterer-Siwe disease and Schuller-Christian disease as related manifestations of a single nosologic entity. Arch Pathol. 1953;56:84–102.

    CAS  Google Scholar 

  3. Birbeck MS, Breathnach AS, Everall JD. An electron microscopic study of basal melanocytes and high-level clear cells (Langerhans cells) in vitiligo. J Invest Dermatol. 1961;31:51–64.

    Article  Google Scholar 

  4. Writing Group of the Histiocyte Society. Histiocytosis syndromes in children. Lancet. 1987;1:208–9.

    Google Scholar 

  5. Willman CL, Busque L, Griffith BB, et al. Langerhans’-cell histiocytosis (histiocytosis X): a clonal proliferative disease. N Engl J Med. 1994;331:154–60.

    Article  CAS  PubMed  Google Scholar 

  6. Yu RC, Chu C, Buluwela L, Chu AC. Clonal proliferation of Langerhans cells in Langerhans cell histiocytosis. Lancet. 1994;343:767–8.

    Article  CAS  PubMed  Google Scholar 

  7. Allen CE, Li L, Peters TL, et al. Cell-specific gene expression in Langerhans cell histiocytosis lesions reveals a distinct profile compared with epidermal Langerhans cells. J Immunol. 2010;184:4557–67.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. Badalian-Very G, Vergilio J, Degar BA, et al. Recurrent BRAF mutations in Langerhans cell histiocytosis. Blood. 2010;116:1919–23.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  9. Brown NA, Furtado LV, Betz BL, et al. High prevalence of somatic MAP2K1 mutations in BRAF V600E-negative Langerhans cell histiocytosis. Blood. 2014;124:1655–8.

    Article  CAS  PubMed  Google Scholar 

  10. Berres ML, Lim KPH, Peters T, et al. BRAF-V600E expression in precursor versus differentiated dendritic cells defines clinically distinct LCH risk groups. J Exp Med. 2014;211:669–83.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  11. Yousem SA, Colby TV, Chen YY, et al. Pulmonary Langerhans’ cell histiocytosis: molecular analysis of clonality. Am J Surg Pathol. 2001;25:630–6.

    Article  CAS  PubMed  Google Scholar 

  12. Elia D, Torre O, Cassandro R, Caminati A, Harari S. Pulmonary Langerhans cell histiocytosis: a comprehensive analysis of 40 patients and literature review. Eur J Intern Med. 2015;26:351–6.

    Article  PubMed  Google Scholar 

  13. Egeler RM, Neglia JP, Puccetti DM, Brennan CA, Nesbit ME. Association of Langerhans cell histiocytosis with malignant neoplasms. Cancer. 1993;71:865–73.

    Article  CAS  PubMed  Google Scholar 

  14. Richmond I, Eyden BP, Banerjee SS. Intranodal Langerhans’ cell histiocytosis associated with malignant melanoma. Histopathology. 1995;26:380–2.

    Article  CAS  PubMed  Google Scholar 

  15. McClain K, Jin H, Gresik V, Favara B. Langerhans cell histiocytosis: lack of viral etiology. Am J Hematol. 1994;47:16–20.

    Article  CAS  PubMed  Google Scholar 

  16. Arico M, Nichols K, Whitlock JA, et al. Familial clustering of Langerhans cell histiocytosis. Br J Haematol. 1999;107:883–8.

    Article  CAS  PubMed  Google Scholar 

  17. Hamre M, Hedberg J, Buckley J, et al. Langerhans cell histiocytosis: an exploratory epidemiologic study of 177 cases. Med Pediatr Oncol. 1997;28:92–7.

    Article  CAS  PubMed  Google Scholar 

  18. Lieberman PH, Jones CR, Steinman RM, et al. Langerhans cell (eosinophilic) granulomatosis. Am J Surg Pathol. 1996;20:519–52.

    Article  CAS  PubMed  Google Scholar 

  19. Howarth DM, Gilchrist GS, Mullan B, Wiseman GA, Edmonson JH, Schomberg PJ. Langerhans cell histiocytosis: diagnosis, natural history, management, and outcome. Cancer. 1999;85:2278–90.

    Article  CAS  PubMed  Google Scholar 

  20. West DS, Dogan A, Quint PS, et al. Clonally related follicular lymphomas and Langerhans cell neoplasms: expanding the spectrum of transdifferentiation. Am J Surg Pathol. 2013;37:978–86.

    Article  PubMed  Google Scholar 

  21. Madrigal-Martínez-Pereda C, Guerrero-Rodríguez V, Guisado-Moya B, Meniz-Garcia C. Langerhans cell histiocytosis; literature review and descriptive analysis of oral manifestations. Med Oral Patol Oral Cir Bucal. 2009;14:E222–8.

    PubMed  Google Scholar 

  22. Mataix J, Betloch I, Lucas-Costa A, Pérez-Crespo M, Moscardó-Guilleme C. Nail changes in Langerhans cell histiocytosis; a possible marker of multisystem disease. Pediatr Dermatol. 2008;25:247–51.

    Article  PubMed  Google Scholar 

  23. Helm KF, Lookingbill DP, Marks JG. A clinical and pathologic study of histiocytosis X in adults. J Am Acad Dermatol. 1993;29:166–70.

    Article  CAS  PubMed  Google Scholar 

  24. Emile JF, Wechsler J, Brousse N, et al. Langerhans’ cell histiocytosis: definitive diagnosis with the use of monoclonal antibody O10 on routinely paraffin-embedded samples. Am J Surg Pathol. 1995;19:636–41.

    Article  CAS  PubMed  Google Scholar 

  25. Lau SK, Chu PG, Weiss LM. Immunohistochemical expression of langerin in Langerhans cell histiocytosis and non-Langerhans cell histiocytic disorders. Am J Surg Pathol. 2008;32:615–9.

    Article  PubMed  Google Scholar 

  26. Kissenpfennig A, Ait-Yahia S, Clair-Moninot V, et al. Disruption of the langerin/CD207 gene abolishes Birbeck granules without a marked loss of Langerhans cell function. Mol Cell Biol. 2005;25:88–99.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  27. Bhattacharjee P, Glusac EJ. Langerhans cell hyperplasia in scabies: a mimic of Langerhans cell histiocytosis. J Cutan Pathol. 2007;34:716–20.

    Article  PubMed  Google Scholar 

  28. Jokinen CH, Wolgamot GM, Wood BL, Olerud J, Argenyi ZB. Lymphomatoid papulosis with CD1a + dendritic cell hyperplasia, mimicking Langerhans cell histiocytosis. J Cutan Pathol. 2007;34:584–7.

    Article  PubMed  Google Scholar 

  29. The French Langerhans’ Cell Histiocytosis Study Group. A multicentre retrospective survey of Langerhans’ cell histiocytosis: 348 cases observed between 1983 and 1993. Arch Dis Child. 1996;75:17–24.

    Article  Google Scholar 

  30. Arico M, Girschikofsky M, Genereau T, et al. Langerhans cell histiocytosis in adults: report from the International Registry of the Histiocyte Society. Eur J Cancer. 2003;39:2341–8.

    Article  CAS  PubMed  Google Scholar 

  31. Simko SJ, Garmezy B, Abhyankar H, et al. Differentiating skin-limited and multisystem Langerhans cell histiocytosis. J Pediatr. 2014;165:990–6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  32. Minkov M, Grois N, McClain K, et al. Langerhans cell histiocytosis: Histiocyte Society evaluation and treatment guidelines. 2009. www.histiocytesociety.org/document?id=290.

  33. Donadieu J, Rolon MA, Thomas C, et al. Endocrine involvement in pediatric-onset Langerhans’ cell histiocytosis: a population-based study. J Pediatr. 2004;144:344–50.

    Article  PubMed  Google Scholar 

  34. Pardanani A, Phyliky RL, Li CY, Tefferi A. 2-Chlorodeoxyadenosine therapy for disseminated Langerhans cell histiocytosis. Mayo Clin Proc. 2003;78:301–6.

    Article  CAS  PubMed  Google Scholar 

  35. Simko SJ, Tran HD, Jones J, et al. Clofarabine salvage therapy in refractory multifocal histiocytic disorders, including Langerhans cell histiocytosis, juvenile xanthogranuloma, and Rosai-Dorfman disease. Pediatr Blood Cancer. 2014;61:479–87.

    Article  CAS  PubMed  Google Scholar 

  36. Szturz P, Adam Z, Řehák Z, et al. Lenalidomide proved effective in multisystem Langerhans cell histiocytosis. Acta Oncol. 2012;51:412–5.

    Article  CAS  PubMed  Google Scholar 

  37. Clohan G, Barnett Y, Gibson J, Reddel SWR, Barnett MH. Langerhans cell histiocytosis with refractory central nervous system involvement responsive to infliximab. J Neurol Neurosurg Psychiatry. 2012;83:573–5.

    Article  Google Scholar 

  38. Haroche J, Cohen-Aubart F, Emile JF, et al. Dramatic efficacy of vemurafenib in both multisystemic and refractory Erdheim-Chester disease and Langerhans cell histiocytosis harboring the BRAF V600E mutation. Blood. 2013;121:1495–500.

    Article  CAS  PubMed  Google Scholar 

  39. Akkari V, Donadieu J, Piguet C, et al. Hematopoietic stem cell transplantation in patients with severe Langerhans cell histiocytosis and hematological dysfunction: experience of the French Langerhans Cell Study Group. Bone Marrow Transplant. 2003;31:1097–103.

    Article  CAS  PubMed  Google Scholar 

  40. Sheehan MP, Atherton DJ, Broadbent V, et al. Topical nitrogen mustard: an effective treatment for cutaneous Langerhans’ cell histiocytosis. J Pediatr. 1991;119:317–21.

    Article  CAS  PubMed  Google Scholar 

  41. Iwatsuki K, Tsugiki M, Yoshizawa N, Tagikawa M, Yamada M, Shamoto M. The effect of phototherapies on cutaneous lesions of histiocytosis X in the elderly. Cancer. 1986;57:1931–6.

    Article  CAS  PubMed  Google Scholar 

  42. Imafuku S, Shibata S, Tashiro A, Furue M. Cutaneous Langerhans cell histiocytosis in an elderly man successfully treated with narrowband ultraviolet B. Br J Dermatol. 2007;157:1267–304.

    Article  Google Scholar 

  43. Taverna JA, Stefanato CM, Wax FD, Demierre MF. Adult cutaneous Langerhans cell histiocytosis responsive to topical imiquimod. J Am Acad Dermatol. 2006;54:911–3.

    Article  PubMed  Google Scholar 

  44. Thomas L, Ducros B, Secchi T, et al. Successful treatment of adult’s Langerhans cell histiocytosis with thalidomide. Arch Dermatol. 1993;129:1261–4.

    Article  CAS  PubMed  Google Scholar 

  45. Kwong YL, Chan ACL, Chan TK. Widespread skin-limited Langerhans cell histiocytosis: complete remission with interferon α. J Am Acad Dermatol. 1997;36:628–9.

    Article  CAS  PubMed  Google Scholar 

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Author information

Authors and Affiliations

  1. Associate Professor of Dermatology and Pathology, MetroHealth Medical Center Case Western Reserve University School of Medicine, Cleveland, OH, USA

    Stephen C. Somach MD

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  1. Stephen C. Somach MD
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Correspondence to Stephen C. Somach MD .

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Editors and Affiliations

  1. Assistant Professor Department of Dermatology, Case Western Reserve University School of Medicine, Cleveland, Ohio, USA

    David R. Crowe

  2. Dermpath Diagnostics, Tampa, Florida, USA

    Michael Morgan

  3. MetroHealth Medical Center, Cleveland, Ohio, USA

    Stephen Somach

  4. Washington, District of Columbia, USA

    Kara Trapp

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Somach, S.C. (2016). Langerhans Cell Histiocytosis. In: Crowe, D., Morgan, M., Somach, S., Trapp, K. (eds) Deadly Dermatologic Diseases. Springer, Cham. https://doi.org/10.1007/978-3-319-31566-9_3

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  • DOI: https://doi.org/10.1007/978-3-319-31566-9_3

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  • Online ISBN: 978-3-319-31566-9

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