Juvenile Xanthogranuloma and Related Non-LCH Disorders

  • Francesco Ceppi
  • Oussama Abla


JXG is a non-Langerhans cell histiocytosis (non-LCH) characterized by xanthomatous skin lesions that usually resolve spontaneously. In a minority of cases, systemic disease can occur and can be life threatening. Recent whole-exome sequencing of JXG cases did not show the BRAF-V600E mutation, although germline mutations in NF1 and NF2 and PI3KCD mutation have been reported in few patients. The standard treatment for solitary and symptomatic CNS JXG is surgical resection, while patients with multifocal cranial or systemic JXG have been successfully treated with LCH-like therapies such as vinblastine/prednisone or nucleoside analogues. Other JXG-like non-LCH disorders, such as cutaneous JXG-like disorders, xanthoma disseminatum, multicentric reticulohistiocytosis, and solitary reticulohistiocytoma, will also be discussed in this chapter.


Juvenile xanthogranuloma Non-LCH disorders related to JXG Cutaneous JXG-like disorders 


  1. 1.
    Weitzman S, Jaffe R. Uncommon histiocytic disorders: the non-Langerhans cell histiocytoses. Pediatr Blood Cancer. 2005;45(3):256–64.CrossRefPubMedGoogle Scholar
  2. 2.
    Favara BE, Feller AC, Pauli M, Jaffe ES, Weiss LM, Arico M, et al. Contemporary classification of histiocytic disorders. The WHO Committee on Histiocytic/Reticulum Cell proliferations. Reclassification Working group of the Histiocyte Society. Med Pediatr Oncol. 1997;29(3):157–66.CrossRefPubMedGoogle Scholar
  3. 3.
    Chu AC. The confusing state of the histiocytoses. Br J Dermatol. 2000;143(3):475–6.PubMedGoogle Scholar
  4. 4.
    Zelger BW, Cerio R. Xanthogranuloma is the archetype of non-Langerhans cell histiocytoses. Br J Dermatol. 2001;145(2):369–71.CrossRefPubMedGoogle Scholar
  5. 5.
    Janssen D, Harms D. Juvenile xanthogranuloma in childhood and adolescence: a clinicopathologic study of 129 patients from the kiel pediatric tumor registry. Am J Surg Pathol. 2005;29(1):21–8.CrossRefPubMedGoogle Scholar
  6. 6.
    Egeler RM, D’Angio GJ. Langerhans cell histiocytosis. J Pediatr. 1995;127(1):1–11.CrossRefPubMedGoogle Scholar
  7. 7.
    Dehner LP. Juvenile xanthogranulomas in the first two decades of life: a clinicopathologic study of 174 cases with cutaneous and extracutaneous manifestations. Am J Surg Pathol. 2003;27(5):579–93.CrossRefPubMedGoogle Scholar
  8. 8.
    Patrizi A, Neri I, Bianchi F, Guerrini V, Misciali C, Paone G, et al. Langerhans cell histiocytosis and juvenile xanthogranuloma. Two case reports. Dermatology (Basel, Switzerland). 2004;209(1):57–61.CrossRefGoogle Scholar
  9. 9.
    Helwig EAHV. Juvenile xanthogranuloma (nevoxanthoendothelioma) proceedings of the American Association of Pathologists and Bacteriologists. Am J Pathol. 1954;30(3):625–6.Google Scholar
  10. 10.
    Zaba LC, Fuentes-Duculan J, Steinman RM, Krueger JG, Lowes MA. Normal human dermis contains distinct populations of CD11c+BDCA-1+ dendritic cells and CD163+FXIIIA+ macrophages. J Clin Invest. 2007;117(9):2517–25.CrossRefPubMedPubMedCentralGoogle Scholar
  11. 11.
    Kraus MD, Haley JC, Ruiz R, Essary L, Moran CA, Fletcher CD. “Juvenile” xanthogranuloma: an immunophenotypic study with a reappraisal of histogenesis. Am J Dermatopathol. 2001;23(2):104–11.CrossRefPubMedGoogle Scholar
  12. 12.
    Chakraborty R, Hampton OA, Shen X, Simko SJ, Shih A, Abhyankar H, et al. Mutually exclusive recurrent somatic mutations in MAP2K1 and BRAF support a central role for ERK activation in LCH pathogenesis. Blood. 2014;124(19):3007–15.CrossRefPubMedPubMedCentralGoogle Scholar
  13. 13.
    Zvulunov A, Barak Y, Metzker A. Juvenile xanthogranuloma, neurofibromatosis, and juvenile chronic myelogenous leukemia. World statistical analysis. Arch Dermatol. 1995;131(8):904–8.CrossRefPubMedGoogle Scholar
  14. 14.
    Diamond EL, Durham BH, Haroche J, Yao Z, Ma J, Parikh SA, et al. Diverse and targetable kinase alterations drive histiocytic neoplasms. Cancer Discov. 2016;6(2):154–65.CrossRefPubMedGoogle Scholar
  15. 15.
    Flaitz C, Allen C, Neville B, Hicks J. Juvenile xanthogranuloma of the oral cavity in children: a clinicopathologic study. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2002;94(3):345–52.CrossRefPubMedGoogle Scholar
  16. 16.
    Chang MW. Update on juvenile xanthogranuloma: unusual cutaneous and systemic variants. Semin Cutan Med Surg. 1999;18(3):195–205.CrossRefPubMedGoogle Scholar
  17. 17.
    Freyer DR, Kennedy R, Bostrom BC, Kohut G, Dehner LP. Juvenile xanthogranuloma: forms of systemic disease and their clinical implications. J Pediatr. 1996;129(2):227–37.CrossRefPubMedGoogle Scholar
  18. 18.
    Papadakis V, Volonaki E, Katsibardi K, Stefanaki K, Valari M, Anagnostakou M, et al. A rare case of neonatal systemic xanthogranulomatosis with severe hepatic disease and metachronous skin involvement. J Pediatr Hematol Oncol. 2012;34(3):226–8.CrossRefPubMedGoogle Scholar
  19. 19.
    Niu L, Zhang C, Meng F, Cai R, Bi Y, Wang Y, et al. Ocular juvenile xanthogranuloma. Optom Vis Sci (official publication of the American Academy of Optometry). 2015;92(6):e126–33.Google Scholar
  20. 20.
    Wang B, Jin H, Zhao Y, Ma J. The clinical diagnosis and management options for intracranial juvenile xanthogranuloma in children: based on four cases and another 39 patients in the literature. Acta Neurochir. 2016;158:1289–97.CrossRefPubMedGoogle Scholar
  21. 21.
    Tamir I, Davir R, Fellig Y, Weintraub M, Constantini S, Spektor S. Solitary juvenile xanthogranuloma mimicking intracranial tumor in children. J Clin Neurosci (official Journal of the Neurosurgical Society of Australasia). 2013;20(1):183–8.CrossRefGoogle Scholar
  22. 22.
    Kwak ES, Marrero DE, Pfannl R, Erbay SH. Sterile brain abscess due to juvenile xanthogranuloma: DWI characteristics. J Neuroimag (official Journal of the American Society of Neuroimaging). 2013;23(3):437–40.CrossRefGoogle Scholar
  23. 23.
    Lesniak MS, Viglione MP, Weingart J. Multicentric parenchymal xanthogranuloma in a child: case report and review of the literature. Neurosurgery. 2002;51(6):1493–8. discussion 8.CrossRefPubMedGoogle Scholar
  24. 24.
    Ashley WW Jr, Narayan P, Park TS, Tu PH, Perry A, Leonard JR. Incidental pediatric intraparenchymal xanthogranuloma: case report and review of the literature. J Neurosurg. 2005;102(3 Suppl):307–10.PubMedGoogle Scholar
  25. 25.
    Nakatani T, Morimoto A, Kato R, Tokuda S, Sugimoto T, Tokiwa K, et al. Successful treatment of congenital systemic juvenile xanthogranuloma with Langerhans cell histiocytosis-based chemotherapy. J Pediatr Hematol Oncol. 2004;26(6):371–4.CrossRefPubMedGoogle Scholar
  26. 26.
    Dolken R, Weigel S, Schroder H, Hartwig M, Harms D, Beck JF. Treatment of severe disseminated juvenile systemic xanthogranuloma with multiple lesions in the central nervous system. J Pediatr Hematol Oncol. 2006;28(2):95–7.CrossRefPubMedGoogle Scholar
  27. 27.
    Orsey A, Paessler M, Lange BJ, Nichols KE. Central nervous system juvenile xanthogranuloma with malignant transformation. Pediatr Blood Cancer. 2008;50(4):927–30.CrossRefPubMedGoogle Scholar
  28. 28.
    Longmuir S, Dumitrescu A, Kwon Y, Boldt HC, Hong S. Juvenile xanthogranulomatosis with bilateral and multifocal ocular lesions of the iris, corneal scleral limbus, and choroid. J AAPOS (the official publication of the American Association for Pediatric Ophthalmology and Strabismus / American Association for Pediatric Ophthalmology and Strabismus). 2011;15(6):598–600.CrossRefGoogle Scholar
  29. 29.
    Ashkenazy N, Henry CR, Abbey AM, McKeown CA, Berrocal AM, Murray TG. Successful treatment of juvenile xanthogranuloma using bevacizumab. J AAPOS (the official publication of the American Association for Pediatric Ophthalmology and Strabismus / American Association for Pediatric Ophthalmology and Strabismus). 2014;18(3):295–7.CrossRefGoogle Scholar
  30. 30.
    Rajendra B, Duncan A, Parslew R, Pizer BL. Successful treatment of central nervous system juvenile xanthogranulomatosis with cladribine. Pediatr Blood Cancer. 2009;52(3):413–5.CrossRefPubMedGoogle Scholar
  31. 31.
    Auvin S, Cuvellier JC, Vinchon M, Defoort-Dhellemes S, Soto-Ares G, Nelken B, et al. Subdural effusion in a CNS involvement of systemic juvenile xanthogranuloma: a case report treated with vinblastin. Brain Dev. 2008;30(2):164–8.CrossRefPubMedGoogle Scholar
  32. 32.
    Stover DG, Alapati S, Regueira O, Turner C, Whitlock JA. Treatment of juvenile xanthogranuloma. Pediatr Blood Cancer. 2008;51(1):130–3.CrossRefPubMedGoogle Scholar
  33. 33.
    Yamada K, Yasui M, Sawada A, Inoue M, Nakayama M, Kawa K. Severe persistent bone marrow failure following therapy with 2-chlorodeoxyadenosine for relapsing juvenile xanthogranuloma of the brain. Pediatr Blood Cancer. 2012;58(2):300–2.CrossRefPubMedGoogle Scholar
  34. 34.
    Simko SJ, Tran HD, Jones J, Bilgi M, Beaupin LK, Coulter D, 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(3):479–87.CrossRefPubMedGoogle Scholar
  35. 35.
    Tahan SR, Pastel-Levy C, Bhan AK, Mihm MC Jr. Juvenile xanthogranuloma. Clinical and pathologic characterization. Arch Pathol Lab Med. 1989;113(9):1057–61.PubMedGoogle Scholar
  36. 36.
    Asarch A, Thiele JJ, Ashby-Richardson H, Norden PS. Cutaneous disseminated xanthogranuloma in an adult: case report and review of the literature. Cutis. 2009;83(5):243–9.PubMedGoogle Scholar
  37. 37.
    Namiki T, Miura K, Yokozeki H. Multiple CD163(+) adult xanthogranuloma associated with myelodysplastic syndrome. J Dermatol. 2015;42(11):1106–7.CrossRefPubMedGoogle Scholar
  38. 38.
    Shoo BA, Shinkai K, McCalmont TH, Fox LP. Xanthogranulomas associated with hematologic malignancy in adulthood. J Am Acad Dermatol. 2008;59(3):488–93.CrossRefPubMedGoogle Scholar
  39. 39.
    Chisolm SS, Schulman JM, Fox LP. Adult xanthogranuloma, reticulohistiocytosis, and Rosai-Dorfman disease. Dermatol Clin. 2015;33(3):465–72; discussion 73.Google Scholar
  40. 40.
    Lange M, Izycka-Swieszewska E, Michajlowski I, Baranska-Rybak W. Benign cephalic histiocytosis. Cutis. 2015;95(6):E15–7.PubMedGoogle Scholar
  41. 41.
    Gianotti F, Caputo R, Ermacora E. Singular “infantile histiocytosis with cells with intracytoplasmic vermiform particles”. Bull Soc Fr Dermatol Syphiligr. 1971;78(3):232–3.PubMedGoogle Scholar
  42. 42.
    Weston WL, Travers SH, Mierau GW, Heasley D, Fitzpatrick J. Benign cephalic histiocytosis with diabetes insipidus. Pediatr Dermatol. 2000;17(4):296–8.CrossRefPubMedGoogle Scholar
  43. 43.
    Jang KA, Ahn SJ, Choi JH, Sung KJ, Moon KC, Koh JK. Histiocytic disorders with spontaneous regression in infancy. Pediatr Dermatol. 2000;17(5):364–8.CrossRefPubMedGoogle Scholar
  44. 44.
    Jang KA, Lee HJ, Choi JH, Sung KJ, Koh JK, Moon KC. Generalized eruptive histiocytoma of childhood. Br J Dermatol. 1999;140(1):174–6.CrossRefPubMedGoogle Scholar
  45. 45.
    Wee SH, Kim HS, Chang SN, Kim DK, Park WH. Generalized eruptive histiocytoma: a pediatric case. Pediatr Dermatol. 2000;17(6):453–5.CrossRefPubMedGoogle Scholar
  46. 46.
    Chern E, Yau D, Ho JC. Generalized eruptive histiocytosis in an infant. Acta Derm Venereol. 2010;90(2):204–5.CrossRefPubMedGoogle Scholar
  47. 47.
    Misery L, Kanitakis J, Hermier C, Cambazard F. Generalized eruptive histiocytoma in an infant with healing in summer: long-term follow-up. Br J Dermatol. 2001;144(2):435–7.CrossRefPubMedGoogle Scholar
  48. 48.
    Lan Ma H, Metze D, Luger TA, Steinhoff M. Successful treatment of generalized eruptive histiocytoma with PUVA. J Dtsch Dermatol Ges. 2007;5(2):131–4.CrossRefPubMedGoogle Scholar
  49. 49.
    Shon W, Peters MS, Reed KB, Ketterling RP, Dogan A, Gibson LE. Atypical generalized eruptive histiocytosis clonally related to chronic myelomonocytic leukemia with loss of Y chromosome. J Cutan Pathol. 2013;40(8):725–9.CrossRefPubMedGoogle Scholar
  50. 50.
    Montero I, Gutierrez-Gonzalez E, Ginarte M, Toribio J. Generalized eruptive histiocytosis in a patient with chronic myelomonocytic leukemia. Actas Dermosifiliogr. 2012;103(7):643–4.CrossRefPubMedGoogle Scholar
  51. 51.
    Klemke CD, Dippel E, Geilen CC, Koenigsmann MP, Thiel E, Orfanos CE, et al. Atypical generalized eruptive histiocytosis associated with acute monocytic leukemia. J Am Acad Dermatol. 2003;49(5 Suppl):S233–6.CrossRefPubMedGoogle Scholar
  52. 52.
    Statham BN, Fairris GM, Cotterill JA. Atypical eruptive histiocytosis – a marker of underlying malignancy? Br J Dermatol. 1984;110(1):103–5.CrossRefPubMedGoogle Scholar
  53. 53.
    Ziegler B, Peitsch WK, Reiter A, Marx A, Goerdt S, Geraud C. Generalized eruptive Histiocytosis associated with FIP1L1-PDGFRA-positive chronic eosinophilic leukemia. JAMA Dermatol. 2015;151(7):766–9.CrossRefPubMedGoogle Scholar
  54. 54.
    Zelger BW, Staudacher C, Orchard G, Wilson-Jones E, Burgdorf WH. Solitary and generalized variants of spindle cell xanthogranuloma (progressive nodular histiocytosis). Histopathology. 1995;27(1):11–9.CrossRefPubMedGoogle Scholar
  55. 55.
    Taunton OD, Yeshurun D, Jarratt M. Progressive nodular histiocytoma. Arch Dermatol. 1978;114(10):1505–8.CrossRefPubMedGoogle Scholar
  56. 56.
    Nofal A, Assaf M, Tawfik A, Elsayed N, Nofal E, Elnakib N, et al. Progressive nodular histiocytosis: a case report and literature review. Int J Dermatol. 2011;50(12):1546–51.CrossRefPubMedGoogle Scholar
  57. 57.
    Glavin FL, Chhatwall H, Karimi K. Progressive nodular histiocytosis: a case report with literature review, and discussion of differential diagnosis and classification. J Cutan Pathol. 2009;36(12):1286–92.CrossRefPubMedGoogle Scholar
  58. 58.
    Gonzalez Ruiz A, Bernal Ruiz AI, Aragoneses Fraile H, Peral Martinez I, Garcia MM. Progressive nodular histiocytosis accompanied by systemic disorders. Br J Dermatol. 2000;143(3):628–31.CrossRefPubMedGoogle Scholar
  59. 59.
    Watanabe T, Watanabe D, Tamada Y, Matsumoto Y. Progressive nodular histiocytosis – a five-year follow up. Eur J Dermatol. 2008;18(2):200–2.PubMedGoogle Scholar
  60. 60.
    Vadoud-Seyedi J, Vadoud-Seyedi R, De Dobbeleer G. Progressive nodular histiocytomas. Br J Dermatol. 2000;143(3):678–9.CrossRefPubMedGoogle Scholar
  61. 61.
    Szekeres E, Tiba A, Korom I. Xanthoma disseminatum: a rare condition with non-X, non-lipid cutaneous histiocytopathy. J Dermatol Surg Oncol. 1988;14(9):1021–4.CrossRefPubMedGoogle Scholar
  62. 62.
    Zelger B, Burgdorf WH. The cutaneous “histiocytoses”. Adv Dermatol. 2001;17:77–114.PubMedGoogle Scholar
  63. 63.
    Nanda A, Kanwar AJ, Kapoor MM, Radotra BD, Kaur S. Xanthoma disseminatum. Int J Dermatol. 1990;29(10):727–8.CrossRefPubMedGoogle Scholar
  64. 64.
    Coldiron BM, Cruz PD Jr, Freeman RG, Sontheimer RD. Benign non-X histiocytosis: a unique case bridging several of the non-X histiocytic syndromes. J Am Acad Dermatol. 1988;18(6):1282–9.CrossRefPubMedGoogle Scholar
  65. 65.
    Ferrando J, Campo-Voegeli A, Soler-Carrillo J, Munoz E, Sole M, Palou J, et al. Systemic xanthohistiocytoma: a variant of xanthoma disseminatum? Br J Dermatol. 1998;138(1):155–60.CrossRefPubMedGoogle Scholar
  66. 66.
    Oka M, Oniki S, Komatsu M, Ikeda T, Matsuo M, Miyamoto Y, et al. Xanthoma disseminatum with intracranial involvement: case report and literature review. Int J Dermatol. 2010;49(2):193–9.CrossRefPubMedGoogle Scholar
  67. 67.
    Hammond RR, Mackenzie IR. Xanthoma disseminatum with massive intracranial involvement. Clin Neuropathol. 1995;14(6):314–21.PubMedGoogle Scholar
  68. 68.
    Buyukavci M, Selimoglu A, Yildirim U, Ertekin V, Atasoy M. Xanthoma disseminatum with hepatic involvement in a child. Pediatr Dermatol. 2005;22(6):550–3.CrossRefPubMedGoogle Scholar
  69. 69.
    Calverly DC, Wismer J, Rosenthal D, de Sa D, Barr RD. Xanthoma disseminatum in an infant with skeletal and marrow involvement. J Pediatr Hematol Oncol. 1995;17(1):61–5.CrossRefPubMedGoogle Scholar
  70. 70.
    Eisendle K, Linder D, Ratzinger G, Zelger B, Philipp W, Piza H, et al. Inflammation and lipid accumulation in xanthoma disseminatum: therapeutic considerations. J Am Acad Dermatol. 2008;58(2 Suppl):S47–9.CrossRefPubMedGoogle Scholar
  71. 71.
    Khezri F, Gibson LE, Tefferi A. Xanthoma disseminatum: effective therapy with 2-chlorodeoxyadenosine in a case series. Arch Dermatol. 2011;147(4):459–64.CrossRefPubMedGoogle Scholar
  72. 72.
    Kim WJ, Ko HC, Kim BS, Kim MB. Successful treatment of xanthoma disseminatum with combined lipid lowering agents. Ann Dermatol. 2012;24(3):380–2.CrossRefPubMedPubMedCentralGoogle Scholar
  73. 73.
    Lee EH, Kang TW, Kim SC. Successful treatment of xanthoma disseminatum with simvastatin. J Dermatol. 2011;38(10):1015–7.CrossRefPubMedGoogle Scholar
  74. 74.
    Park M, Boone B, Devos S. Xanthoma disseminatum: case report and mini-review of the literature. Acta Dermatovenerol Croat. 2014;22(2):150–4.PubMedGoogle Scholar
  75. 75.
    Pruvost C, Picard-Dahan C, Bonnefond B, Grossin M, Gehanno P, Souteyrand P, et al. Vinblastine treatment for extensive non-X histiocytosis (xanthoma disseminatum). Annales de dermatologie et de venereologie. 2004;131(3):271–3.CrossRefPubMedGoogle Scholar
  76. 76.
    Seaton ED, Pillai GJ, Chu AC. Treatment of xanthoma disseminatum with cyclophosphamide. Br J Dermatol. 2004;150(2):346–9.CrossRefPubMedGoogle Scholar
  77. 77.
    Zinoun M, Hali F, Marnissi F, Lazaar S, Benchikhi H. Xanthoma disseminatum with asymptomatic multisystem involvement. Annales de dermatologie et de venereologie. 2015;142(4):276–80.CrossRefPubMedGoogle Scholar
  78. 78.
    Campero M, Campero S, Guerrero J, Aouba A, Castro A. Cerebral and cutaneous involvements of xanthoma disseminatum successfully treated with an interleukin-1 receptor antagonist: a case report and minireview. Dermatology (Basel, Switzerland). 2016;232(2):171–6.CrossRefGoogle Scholar
  79. 79.
    Jaffe R. The histiocytoses. Clin Lab Med. 1999;19(1):135–55.PubMedGoogle Scholar
  80. 80.
    Outland JD, Keiran SJ, Schikler KN, Callen JP. Multicentric reticulohistiocytosis in a 14-year-old girl. Pediatr Dermatol. 2002;19(6):527–31.CrossRefPubMedGoogle Scholar
  81. 81.
    Tariq S, Hugenberg ST, Hirano-Ali SA, Tariq H. Multicentric reticulohistiocytosis (MRH): case report with review of literature between 1991 and 2014 with in depth analysis of various treatment regimens and outcomes. SpringerPlus. 2016;5:180.CrossRefPubMedPubMedCentralGoogle Scholar
  82. 82.
    Havill S, Duffill M, Rademaker M. Multicentric reticulohistiocytosis in a child. Australas J Dermatol. 1999;40(1):44–6.CrossRefPubMedGoogle Scholar
  83. 83.
    Raphael SA, Cowdery SL, Faerber EN, Lischner HW, Schumacher HR, Tourtellotte CD. Multicentric reticulohistiocytosis in a child. J Pediatr. 1989;114(2):266–9.CrossRefPubMedGoogle Scholar
  84. 84.
    Jha VK, Kumar R, Kunwar A, Singh A, Kumar M, Kumar M, et al. Efficacy of vinblastine and prednisone in multicentric reticulohistiocytosis with onset in infancy. Pediatrics. 2016;137(6):e20152118.CrossRefPubMedGoogle Scholar
  85. 85.
    Gorman JD, Danning C, Schumacher HR, Klippel JH, Davis JC Jr. Multicentric reticulohistiocytosis: case report with immunohistochemical analysis and literature review. Arthritis Rheum. 2000;43(4):930–8.CrossRefPubMedGoogle Scholar
  86. 86.
    Campbell DA, Edwards NL. Multicentric reticulohistiocytosis: systemic macrophage disorder. Baillieres Clin Rheumatol. 1991;5(2):301–19.CrossRefPubMedGoogle Scholar
  87. 87.
    Luz FB, Gaspar TAP, Kalil-Gaspar N, Ramos-e-Silva M. Multicentric reticulohistiocytosis. J Eur Acad Dermatol Venereol. 2001;15(6):524–31.CrossRefPubMedGoogle Scholar
  88. 88.
    Liang GC, Granston AS. Complete remission of multicentric reticulohistiocytosis with combination therapy of steroid, cyclophosphamide, and low-dose pulse methotrexate. Case report, review of the literature, and proposal for treatment. Arthritis Rheum. 1996;39(1):171–4.CrossRefPubMedGoogle Scholar
  89. 89.
    Jansen TL, Kruithof IG. Diagnostic image (141) nodules in a patient with rheumatoid arthritis. Multicentric reticulohistiocytosis as a paraneoplastic manifestation. Ned Tijdschr Geneeskd. 2003;147(22):1067.PubMedGoogle Scholar
  90. 90.
    Olson J, Mann JA, White K, Cartwright VW, Bauer J, Nolt D. Multicentric reticulohistiocytosis: a case report of an atypical presentation in a 2-year-old. Pediatr Dermatol. 2015;32(3):e70–3.CrossRefPubMedGoogle Scholar
  91. 91.
    Aouba A, Leclerc-Mercier S, Fraitag S, Martin-Silva N, Bienvenu B, Georgin-Lavialle S. Assessment and effective targeting of interleukin-1 in multicentric reticulohistyocytosis. Joint Bone Spine. 2015;82(4):280–3.CrossRefPubMedGoogle Scholar
  92. 92.
    Pacheco-Tena C, Reyes-Cordero G, Ochoa-Albiztegui R, Rios-Barrera V, Gonzalez-Chavez SA. Treatment of multicentric reticulohistiocytosis with tocilizumab. J Clin Rheumatol (Practical Reports on Rheumatic & Musculoskeletal Diseases). 2013;19(5):272–6.CrossRefGoogle Scholar
  93. 93.
    Yeter KC, Arkfeld DG. Treatment of multicentric reticulohistiocytosis with adalimumab, minocycline, methotrexate. Int J Rheum Dis. 2013;16(1):105–6.CrossRefPubMedGoogle Scholar
  94. 94.
    Chalom EC, Rosenstein ED, Kramer N. Cyclosporine as a treatment for multicentric reticulohistiocytosis. J Rheumatol. 2000;27(2):556.PubMedGoogle Scholar
  95. 95.
    Savasan S, Smith L, Scheer, Dansey R, Abella E. Successful bone marrow transplantation for life threating xanthogranuloma disseminatum in neurofibromatosis type-1. Pediatr Transplant 2005;9:534–6.Google Scholar
  96. 96.
    Bastida J, Camacho-Galan R, Armesto-Fernandez MA, Diaz-CascajoC. Adult disseminated primary papular xanthoma treated with doxycycline. Arch Dermatol.Google Scholar

Copyright information

© Springer International Publishing AG 2018

Authors and Affiliations

  1. 1.Department Woman-Mother-Child, Divisions of Pediatric Oncology and HematologyUniversity Hospital of LausanneLausanneSwitzerland
  2. 2.Department of Pediatrics, Division of Hematology/OncologyHospital for Sick Children, University of TorontoTorontoCanada

Personalised recommendations