Systemic therapy of necrobiotic xanthogranuloma: a systematic review (2024)

1. Kossard S, Winkelmann RK. Necrobiotic xanthogranuloma. Australas J Dermatol. 1980;21:85–88. [PubMed] [Google Scholar]

2. Chave TA, Chowdhury MM, Holt PJ. Recalcitrant necrobiotic xanthogranuloma responding to pulsed high-dose oral dexamethasone plus maintenance therapy with oral prednisolone. Br J Dermatol. 2001;144:158–161. [PubMed] [Google Scholar]

3. Silapunt S, Chon SY. Generalized necrobiotic xanthogranuloma successfully treated with lenalidomide. J Drugs Dermatol. 2010;9:273–276. [PubMed] [Google Scholar]

4. Dholaria BR, Cappel M, Roy V. Necrobiotic xanthogranuloma associated with monoclonal gammopathy: successful treatment with lenalidomide and dexamethasone. Ann Hematol. 2016;95:671–672. [PubMed] [Google Scholar]

5. Gun D, Demircay Z, Demirkesen C. Necrobiotic xanthogranuloma in a burn scar. Int J Dermatol. 2004;43:293–295. [PubMed] [Google Scholar]

6. Umbert I, Winkelmann RK. Necrobiotic xanthogranuloma with cardiac involvement. Br J Dermatol. 1995;133:438–443. [PubMed] [Google Scholar]

7. Hunter L, Burry AF. Necrobiotic xanthogranuloma: a systemic disease with paraproteinemia. Pathology. 1985;17:533–536. [PubMed] [Google Scholar]

8. Frank SB, Weidman AI. Xanthoma disseminatum; an unusual form with extension of xanthomatous changes into muscle. AMA Arch Derm Syphilol. 1952;65:88–94. [PubMed] [Google Scholar]

9. Novak PM, Robbins TO, Winkelmann RK. Necrobiotic xanthogranuloma with myocardial lesions and nodular transformation of the liver. Hum Pathol. 1992;23:195–196. [PubMed] [Google Scholar]

10. Winkelmann RK, Litzow MR, Umbert IJ, Lie JT. Giant cell granulomatous pulmonary and myocardial lesions in necrobiotic xanthogranuloma with paraproteinemia. Mayo Clin Proc. 1997;72:1028–1033. [PubMed] [Google Scholar]

11. Mehregan DA, Winkelmann RK. Necrobiotic xanthogranuloma. Arch Dermatol. 1992;128:94–100. [PubMed] [Google Scholar]

12. Spicknall KE, Mehregan DA. Necrobiotic xanthogranuloma. Int J Dermatol. 2009;48:1–10. [PubMed] [Google Scholar]

13. Rose A, Robinson M, Kamino H, Latkowski JA. Necrobiotic xanthogranuloma. Dermatol Online J. 2012;18:30. [PubMed] [Google Scholar]

14. Wood AJ, Wagner MV, Abbott JJ, Gibson LE. Necrobiotic xanthogranuloma: a review of 17 cases with emphasis on clinical and pathologic correlation. Arch Dermatol. 2009;145:279–284. [PubMed] [Google Scholar]

15. Hallermann C, Tittelbach J, Norgauer J, Ziemer M. Successful treatment of necrobiotic xanthogranuloma with intravenous immunoglobulin. Arch Dermatol. 2010;146:957–960. [PubMed] [Google Scholar]

16. Miguel D, Lukacs J, Illing T, Elsner P. Treatment of necrobiotic xanthogranuloma—a systematic review. J Eur Acad Dermatol Venereol. 2017;31:221–235. [PubMed] [Google Scholar]

17. Chang SE, Lee WS, Lee MW, Choi JH, Sung KJ, Moon KC, Koh JK. A case of necrobiotic xanthogranuloma without paraproteinemia presenting as a solitary tumor on the thigh. Int J Dermatol. 2003;42:470–472. [PubMed] [Google Scholar]

18. Codere F, Lee RD, Anderson RL. Necrobiotic xanthogranuloma of the eyelid. Arch Ophthalmol. 1983;101:60–63. [PubMed] [Google Scholar]

19. Criado PR, Vasconcellos C, Pegas JR, Lopes LF, Ramos CF, Tebcherani AJ, Valente NY. Necrobiotic xanthogranuloma with lambda paraproteinemia: case report of successful treatment with melphalan and prednisone. J Dermatolog Treat. 2002;13:87–89. [PubMed] [Google Scholar]

20. Criton S, Asokan PU, Pailey S, Kuttappan SS, Rodriguez FP. Necrobiotic xanthogranuloma with paraproteinaemia. Indian J Dermatol Venereol Leprol. 1996;62:383–385. [PubMed] [Google Scholar]

21. Efebera Y, Blanchard E, Allam C, Han A, Lee S, Munshi N. Complete response to thalidomide and dexamethasone in a patient with necrobiotic xanthogranuloma associated with monoclonal gammopathy: a case report and review of the literature. Clin Lymphoma Myeloma Leuk. 2011;11:298–302. [PMC free article] [PubMed] [Google Scholar]

22. Finan MC, Winkelmann RK. Necrobiotic xanthogranuloma with paraproteinemia. A review of 22 cases. Medicine (Baltimore) 1986;65:376–388. [PubMed] [Google Scholar]

23. Finelli LG, Ratz JL. Plasmapheresis, a treatment modality for necrobiotic xanthogranuloma. J Am Acad Dermatol. 1987;17:351–354. [PubMed] [Google Scholar]

24. Flann S, Wain EM, Halpern S, Andrews V, Whittaker S. Necrobiotic xanthogranuloma with paraproteinaemia. Clin Exp Dermatol. 2006;31:248–251. [PubMed] [Google Scholar]

25. Gacto P, Barrera F, Pereyra JJ, Fernandez-Ortega P. Necrobiotic xanthogranuloma: efficacy of surgery in 2 patients. Actas Dermosifiliogr. 2009;100:499–502. [PubMed] [Google Scholar]

26. Ghani S, Al Ustwani O, Khalid B, Bogner P, Grassi M, Powell J, Bhat SA. Periorbital necrobiotic xanthogranuloma treated successfully with novel multiple myeloma therapy. Clin Adv Hematol Oncol. 2013;11:678–680. [PubMed] [Google Scholar]

27. Ghiasi N, Alavi A, Coutts PM, Ghazarian D, Sibbald RG. Necrobiotic xanthogranuloma as an unusual cause of refractive chronic bilateral leg ulceration. Int J Low Extrem Wounds. 2012;11:293–295. [PubMed] [Google Scholar]

28. Goede JS, Misselwitz B, Taverna C, Schanz U, Dispenzieri A, Hummel Y, Trueb RM, Fehr J. Necrobiotic xanthogranuloma successfully treated with autologous stem cell transplantation. Ann Hematol. 2007;86:303–306. [PubMed] [Google Scholar]

29. Hauser C, Schifferli J, Saurat JH. Complement consumption in a patient with necrobiotic xanthogranuloma and paraproteinemia. J Am Acad Dermatol. 1991;24:908–911. [PubMed] [Google Scholar]

30. Holden CA, Winkelmann RK, Wilson Jones E. Necrobiotic xanthogranuloma: a report of four cases. Br J Dermatol. 1986;114:241–250. [PubMed] [Google Scholar]

31. Kawakami Y, Yamamoto T. Letter: necrobiotic xanthogranuloma of extremities in an elderly patient successfully treated with low-dose prednisolone. Dermatol Online J. 2011;17:13. [PubMed] [Google Scholar]

32. Khan IJ, Azam NA, Sullivan SC, Habboush HW, Christian A. Necrobiotic xanthogranuloma successfully treated with a combination of dexamethasone and oral cyclophosphamide. Can J Ophthalmol. 2009;44:335–336. [PubMed] [Google Scholar]

33. Liszewski W, Wisniewski JD, Safah H, Boh EE. Treatment of refractory necrobiotic xanthogranulomas with extracorporeal photopheresis and intravenous immunoglobulin. Dermatol Ther. 2014;27:268–271. [PubMed] [Google Scholar]

34. Luck J, Layton A, Noble BA. Necrobiotic xanthogranuloma with orbital involvement. J R Soc Med. 1992;85:357–358. [PMC free article] [PubMed] [Google Scholar]

35. Macfarlane AW, Verbov JL. Necrobiotic xanthogranuloma with paraproteinaemia. Br J Dermatol. 1985;113:339–343. [PubMed] [Google Scholar]

36. Machado S, Alves R, Lima M, Leal I, Massa A. Cutaneous necrobiotic xanthogranuloma (NXG)-successfully treated with low dose chlorambucil. Eur J Dermatol. 2001;11:458–462. [PubMed] [Google Scholar]

37. Meyer S, Szeimies RM, Landthaler M, Hohenleutner S. Cyclophosphamide-dexamethasone pulsed therapy for treatment of recalcitrant necrobiotic xanthogranuloma with paraproteinemia and ocular involvement. Br J Dermatol. 2005;153:443–445. [PubMed] [Google Scholar]

38. Naghashpour M, Setoodeh R, Moscinski L, Bergier G, McCardle T, Glass F, Sokol L. Nonnecrobiotic necrobiotic xanthogranuloma as an initial manifestation of paraproteinemia and small lymphocytic lymphoma in a patient with Sjogren syndrome. Am J Dermatopathol. 2011;33:855–857. [PubMed] [Google Scholar]

39. Oumeish OY, Oumeish I, Tarawneh M, Salman T, Sharaiha A. Necrobiotic xanthogranuloma associated with paraproteinemia and non-Hodgkin's lymphoma developing into chronic lymphocytic leukemia: the first case reported in the literature and review of the literature. Int J Dermatol. 2006;45:306–310. [PubMed] [Google Scholar]

40. Plotnick H, Taniguchi Y, Hashimoto K, Negendank W, Tranchida L. Periorbital necrobiotic xanthogranuloma and stage I multiple myeloma. Ultrastructure and response to pulsed dexamethasone documented by magnetic resonance imaging. J Am Acad Dermatol. 1991;25:373–377. [PubMed] [Google Scholar]

41. Rayner SA, Duncombe AS, Keefe M, Theaker J, Manners RM. Necrobiotic xanthogranuloma occurring in an eyelid scar. Orbit. 2008;27:191–194. [PubMed] [Google Scholar]

42. Reddy VC, Salomao DR, Garrity JA, Baratz KH, Patel SV. Periorbital and ocular necrobiotic xanthogranuloma leading to perforation. Arch Ophthalmol. 2010;128:1493–1494. [PubMed] [Google Scholar]

43. Reeder CB, Connolly SM, Winkelmann RK. The evolution of Hodgkin's disease and necrobiotic xanthogranuloma syndrome. Mayo Clin Proc. 1991;66:1222–1224. [PubMed] [Google Scholar]

44. Rose GE, Patel BC, Garner A, Wright JE. Orbital xanthogranuloma in adults. Br J Ophthalmol. 1991;75:680–684. [PMC free article] [PubMed] [Google Scholar]

45. Rubinstein A, Wolf DJ, Granstein RD. Successful treatment of necrobiotic xanthogranuloma with intravenous immunoglobulin. J Cutan Med Surg. 2013;17:347–350. [PubMed] [Google Scholar]

46. Ryan E, Warren LJ, Szabo F. Necrobiotic xanthogranuloma: response to chlorambucil. Australas J Dermatol. 2012;53:e23–25. [PubMed] [Google Scholar]

47. Saeki H, Tomita M, Kai H, Ohno Y, Le Pavoux A, Kadono T, Tsunemi Y, Sakurai N, Asano Y, Tamaki K. Necrobiotic xanthogranuloma with paraproteinemia successfully treated with melphalan, prednisolone and skin graft. J Dermatol. 2007;34:795–797. [PubMed] [Google Scholar]

48. Shah KC, Poonnoose SI, George R, Jacob M, Rajshekhar V. Necrobiotic xanthogranuloma with cutaneous and cerebral manifestations. Case report and review of the literature. J Neurosurg. 2004;100:1111–1114. [PubMed] [Google Scholar]

49. Spraul CW, Wagner P, Lang GK. Bilateral necrobiotic xanthogranuloma of the eyelids with associated paraproteinemia: case report and review of literature. Klin Monbl Augenheilkd. 2002;219:55–58. [PubMed] [Google Scholar]

50. Sutton L, Sutton S, Sutton M. Treatment of necrobiotic xanthogranuloma with 2-chlorodeoxyadenosine. Skinmed. 2013;11:121–123. [PubMed] [Google Scholar]

51. Szalat R, Pirault J, Fermand JP, Carrie A, Saint-Charles F, Olivier M, Robillard P, Frisdal E, Villard EF, Cathebras P, et al. Physiopathology of necrobiotic xanthogranuloma with monoclonal gammopathy. J Intern Med. 2014;276:269–284. [PMC free article] [PubMed] [Google Scholar]

52. Torabian SZ, Fazel N, Knuttle R. Necrobiotic xanthogranuloma treated with chlorambucil. Dermatol Online J. 2006;12:11. [PubMed] [Google Scholar]

53. Ugurlu S, Bartley GB, Gibson LE. Necrobiotic xanthogranuloma: long-term outcome of ocular and systemic involvement. Am J Ophthalmol. 2000;129:651–657. [PubMed] [Google Scholar]

54. Valentine EA, Friedman HD, Zamkoff KW, Streeten BW. Necrobiotic xanthogranuloma with IgA multiple myeloma: a case report and literature review. Am J Hematol. 1990;35:283–285. [PubMed] [Google Scholar]

55. Venencie PY, Le Bras P, Toan ND, Tchernia G, Delfraissy JF. Recombinant interferon alfa-2b treatment of necrobiotic xanthogranuloma with paraproteinemia. J Am Acad Dermatol. 1995;32:666–667. [PubMed] [Google Scholar]

56. Venencie PY, Puissant A, Verola O, Kerneis Y, Marchat C, Le Bras P, D'Agay MF, Danon F, Valensi F, Turpin G. Necrobiotic xanthogranuloma with myeloma. A case report. Cancer. 1987;59:588–592. [PubMed] [Google Scholar]

57. Wells J, Gillespie R, Zardawi I. Case of recalcitrant necrobiotic xanthogranuloma. Australas J Dermatol. 2004;45:213–215. [PubMed] [Google Scholar]

58. Westermann G, August C, Bonsmann G, Rahn KH, Kisters K. Necrobiotic xanthogranuloma with skin and liver amyloidosis. Med Klin (Munich) 2001;96:50–54. [PubMed] [Google Scholar]

59. Ziemer M, Wedding U, Sander CS, Elsner P. Necrobiotic xanthogranuloma-rapid progression under treatment with melphalan. Eur J Dermatol. 2005;15:363–365. [PubMed] [Google Scholar]

60. Dellatorre G, Miqueloto JK. Necrobiotic xanthogranuloma. JAMA Dermatol. 2020;156:696. [PubMed] [Google Scholar]

61. Gonzales JA, Haemel A, Gross AJ, Acharya NR. Management of uveitis and scleritis in necrobiotic xanthogranuloma. J Ocul Pharmacol Ther. 2017;33:325–333. [PubMed] [Google Scholar]

62. Goyal A, O'Leary D, Vercellotti G, Miller D, McGlave P. Intravenous immunoglobulin for treatment of necrobiotic xanthogranuloma. Dermatol Ther. 2019;32:e12744. [PubMed] [Google Scholar]

63. Guchlerner M, Brockmann MA, Pitz S. Periocular necrobiotic xanthogranuloma with mono- and biclonal gammopathy. Klin Monbl Augenheilkd. 2020;237:41–45. [PubMed] [Google Scholar]

64. Henning C, Meyers S, Swift R, Eades B, Bussell L, Spektor TM, Berenson JR. Efficacy of topical use crisaborole 2% ointment for treatment of necrobiotic xanthogranuloma associated with multiple myeloma. Clin Lymphoma Myeloma Leuk. 2020;20:e492–e495. [PubMed] [Google Scholar]

65. Higgins LS, Go RS, Dingli D, Kumar SK, Rajkumar SV, Dispenzieri A, Buadi FK, Lacy MQ, Lust JA, Kapoor P, et al. Clinical features and treatment outcomes of patients with necrobiotic xanthogranuloma associated with monoclonal gammopathies. Clin Lymphoma Myeloma Leuk. 2016;16:447–452. [PubMed] [Google Scholar]

66. Keorochana N, Klanarongran K, Satayasoontorn K, Chaiamnuay S. Necrobiotic xanthogranuloma scleritis in a case of granulomatosis with polyangiitis (Wegener's granulomatosis) Int Med Case Rep J. 2017;10:323–328. [PMC free article] [PubMed] [Google Scholar]

67. Klingner M, Hansel G, Schonlebe J, Wollina U. Disseminated necrobiotic xanthogranuloma. Hautarzt. 2016;67:902–906. [PubMed] [Google Scholar]

68. Lee HJ, Kim JM, Kim GW, Kim HS, Kim BS, Kim MB, Ko HC. Necrobiotic xanthogranuloma treated with a combination of oral methylprednisolone and cyclosporin. J Dermatol. 2017;44:1190–1191. [PubMed] [Google Scholar]

69. Lopes S, Gomes N, Cesar A, Barros AM, Pinheiro J, Azevedo F. An exuberant case of necrobiotic xanthogranuloma. Indian Dermatol Online J. 2020;11:83–86. [PMC free article] [PubMed] [Google Scholar]

70. Lukacs J, Goetze S, Elsner P. Periocular necrobiotic xanthogranuloma successfully treated with intravenous immunoglobulin. Acta Derm Venereol. 2017;97:754–755. [PubMed] [Google Scholar]

71. Mahendran P, Wee J, Chong H, Natkunarajah J. Necrobiotic xanthogranuloma treated with lenalidomide. Clin Exp Dermatol. 2018;43:345–347. [PubMed] [Google Scholar]

72. Mello RB, Vale E. Necrobiotic xanthogranuloma associated with smoldering multiple myeloma: satisfactory response to cyclophosphamide, dexamethasone, and thalidomide. Bras Dermatol. 2019;94:337–340. [PMC free article] [PubMed] [Google Scholar]

73. Nambudiri VE, McLaughlin C, Lo TC, Zembowicz A, Moschella S. Successful multimodality treatment of recalcitrant necrobiotic xanthogranuloma using electron beam radiation and intravenous immunoglobulin. Clin Exp Dermatol. 2016;41:179–182. [PubMed] [Google Scholar]

74. Nelson CA, Zhong CS, Hashemi DA, Ashchyan HJ, Brown-Joel Z, Noe MH, Imadojemu S, Micheletti RG, Vleugels RA, Wanat KA, et al. A multicenter cross-sectional study and systematic review of necrobiotic xanthogranuloma with proposed diagnostic criteria. JAMA Dermatol. 2020;156:270–279. [PMC free article] [PubMed] [Google Scholar]

75. Nguyen BD. Hepatobiliary and pancreatic: hepatic necrobiotic xanthogranuloma. J Gastroenterol Hepatol. 2017;32:1667. [PubMed] [Google Scholar]

76. Olson RM, Harrison AR, Maltry A, Mokhtarzadeh A. Periorbital necrobiotic xanthogranuloma successfully treated with intravenous immunoglobulin. Case Rep Ophthalmol. 2018;9:70–75. [PMC free article] [PubMed] [Google Scholar]

77. Pedrosa AF, Ferreira O, Calistru A, Mota A, Baudrier T, Sarmento JA, Bettencourt H, Azevedo F. Necrobiotic xanthogranuloma with giant cell hepatitis, successfully treated with intravenous immunoglobulins. Dermatol Ther. 2015;28:68–70. [PubMed] [Google Scholar]

78. Rodriguez O, Meyers C, Weiss BM, Elenitsas R, Rosenbach M. Necrobiotic xanthogranuloma treated with topical nitrogen mustard (Mechlorethamine) JAMA Dermatol. 2016;152:589–590. [PubMed] [Google Scholar]

79. Sagiv O, Thakar SD, Morrell G, Tetzlaff MT, Esmaeli B. Rituximab monotherapy is effective in treating orbital necrobiotic xanthogranuloma. Ophthalmic Plast Reconstr Surg. 2018;34:e24–e27. [PubMed] [Google Scholar]

80. Sfeir JG, Zogala RJ, Popii VB. Hypercalcemia in necrobiotic xanthogranuloma: first reported case and insight into treatment. J Bone Miner Res. 2017;32:784–787. [PubMed] [Google Scholar]

81. Truong K, Venning V, Wain T, Chou S, Fernandez-Penas P. Successful treatment of highly refractory necrobiotic xanthogranuloma with peginterferon alfa-2a. Clin Exp Dermatol. 2020 [PubMed] [Google Scholar]

82. Vu K, Gupta R, Frater J, Atkinson J, Ranganathan P. A 55-year-old man with periorbital and inguinal masses, pericarditis, and pleuritis. Arthritis Care Res (Hoboken) 2017;69:730–736. [PubMed] [Google Scholar]

83. Wei YH, Cheng JJ, Wu YH, Liu CY, Hung CJ, Hsu JD, Hsiao YP. Necrobiotic xanthogranuloma: response to dapsone. Dermatol Ther. 2015;28:7–9. [PubMed] [Google Scholar]

84. Wruhs M, Feldmann R, Sawetz I, Breier F, Steiner A. Necrobiotic xanthogranuloma in a patient with multiple myeloma. Case Rep Dermatol. 2016;8:350–353. [PMC free article] [PubMed] [Google Scholar]

Systemic therapy of necrobiotic xanthogranuloma: a systematic review (2024)


Top Articles
Latest Posts
Article information

Author: Tyson Zemlak

Last Updated:

Views: 5756

Rating: 4.2 / 5 (63 voted)

Reviews: 94% of readers found this page helpful

Author information

Name: Tyson Zemlak

Birthday: 1992-03-17

Address: Apt. 662 96191 Quigley Dam, Kubview, MA 42013

Phone: +441678032891

Job: Community-Services Orchestrator

Hobby: Coffee roasting, Calligraphy, Metalworking, Fashion, Vehicle restoration, Shopping, Photography

Introduction: My name is Tyson Zemlak, I am a excited, light, sparkling, super, open, fair, magnificent person who loves writing and wants to share my knowledge and understanding with you.