Multicentric Reticulohistiocytosis Treatment & Management
- Author: Alisa N Femia, MD; Chief Editor: Herbert S Diamond, MD more...
Because some patients with multicentric reticulohistiocytosis (MRH) may have an underlying malignancy, evaluation for this is important. Patients ought to have at least a good review of systems to direct appropriate additional studies.
No therapy consistently improves MRH. After an average course of 7-8 years, patients often go into remission, but considerable joint destruction may have already occurred. Many different drugs have been used in MRH, but patient response to therapy is difficult to determine because of the rarity of the disease, lack of controlled studies, and tendency for the remission to complicate evaluation of treatment efficacy.
Joint replacement may improve function in patients with burned-out disease that has resulted in deformity. Recently, a case of mutilating arthritis of the small joints of the hands due to MRH was reported as having been successfully managed with arthrodesis of the metacarpophalangeal joints.
In the case of internal malignancies, adequate tumor removal may result in the resolution of histiocytosis.
Mahajan et al reported on the case of a patient with MRH in whom confluent, disfiguring papules on the scalp, forehead, nasolabial folds, retroauricular region, and chin were successfully treated with carbon dioxide laser therapy. According to the authors, complete ablation was achieved, with no recurrence seen over an 8-month follow-up period.
Activity may be limited by the severity of MRH. Physical therapy may prevent deformities and relieve symptoms.
The following consultations may be necessary:
Oncologists or surgeons: May be needed if internal malignancy occurs
Patients with MRH should be monitored at regular intervals to track the activity of the disease and response to therapy.
Although no consistently effective treatment is known for MRH, the associated arthritis may respond to therapy with nonsteroidal anti-inflammatory drugs (NSAIDs).
Systemic corticosteroids, such as prednisone, and/or cytotoxic agents, particularly cyclophosphamide,[5, 6] chlorambucil, and methotrexate,[7, 8, 6, 9] may also affect the inflammatory response, as well as prevent further joint destruction and cause skin lesions to regress. Azathioprine[12, 13] and cyclosporine are also reportedly effective in MRH.
Individual patients have reportedly responded to treatment with alendronate and other bisphosphonates.[15, 16] Antimalarials have also been used in MRH.
Several reports have suggested that combining methotrexate with a tumor necrosis factor (TNF) ̶ alpha antagonist—such as etanercept, infliximab, or adalimumab—is more effective than the use of either alone.[10, 11, 17, 18, 19, 20]
Tocilizumab, an interleukin-6 (IL-6) receptor inhibitor, reportedly caused remission of cutaneous and articular symptoms in a 35-year-old woman whose MRH was refractory to a combination of prednisone and methotrexate.
Sroa N, Zirwas MJ, Bechtel M. Multicentric reticulohistiocytosis: A case report and review of the literature. Cutis. 2010 Mar. 85(3):153-5. [Medline].
Luz FB, Gaspar AP, Ramos-e-Silva M, et al. Immunohistochemical profile of multicentric reticulohistiocytosis. Skinmed. 2005 Mar-Apr. 4(2):71-7. [Medline].
Tashiro A, Takeuchi S, Nakahara T, Oba J, Tsujita J, Fukushi J, et al. Aberrant expression of CD10 in ground-glass-like multinucleated giant cells of multicentric reticulohistiocytosis. J Dermatol. 2010 Nov. 37(11):995-7. [Medline].
Kroot EJ, Weel AE, Hazes JM, Zondervan PE, Heijboer MP, van Daele PL, et al. Diagnostic value of blind synovial biopsy in clinical practice. Rheumatology (Oxford). 2006 Feb. 45(2):192-5. [Medline].
Ginsburg WW, O'Duffy JD, Morris JL, Huston KA. Multicentric reticulohistiocytosis: response to alkylating agents in six patients. Ann Intern Med. 1989 Sep 1. 111(5):384-8. [Medline].
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 Jan. 39(1):171-4. [Medline].
Franck N, Amor B, Ayral X, et al. Multicentric reticulohistiocytosis and methotrexate. J Am Acad Dermatol. 1995 Sep. 33(3):524-5. [Medline].
Gourmelen O, Le Loet X, Fortier-Beaulieu M, et al. Methotrexate treatment of multicentric reticulohistiocytosis. J Rheumatol. 1991 Apr. 18(4):627-8. [Medline].
Rentsch JL, Martin EM, Harrison LC, Wicks IP. Prolonged response of multicentric reticulohistiocytosis to low dose methotrexate. J Rheumatol. 1998 May. 25(5):1012-5. [Medline].
Kovach BT, Calamia KT, Walsh JS, Ginsburg WW. Treatment of multicentric reticulohistiocytosis with etanercept. Arch Dermatol. 2004 Aug. 140(8):919-21. [Medline].
Lovelace K, Loyd A, Adelson D, Crowson N, Taylor JR, Cornelison R. Etanercept and the treatment of multicentric reticulohistiocytosis. Arch Dermatol. 2005 Sep. 141(9):1167-8. [Medline].
Rudd A, Dolianitis C, Varigos G, Howard A. A case of multicentric reticulohistiocytosis responsive to azathioprine in a patient with no underlying malignancy. Australas J Dermatol. 2011 Nov. 52(4):292-4. [Medline].
Hiramanek N, Kossard S, Barnetson RS. Multicentric reticulohistiocytosis presenting with a rash and arthralgia. Australas J Dermatol. 2002 May. 43(2):136-9. [Medline].
Saito K, Fujii K, Awazu Y, et al. A case of systemic lupus erythematosus complicated with multicentric reticulohistiocytosis (MRH): successful treatment of MRH and lupus nephritis with cyclosporin A. Lupus. 2001. 10(2):129-32. [Medline].
Goto H, Inaba M, Kobayashi K, et al. Successful treatment of multicentric reticulohistiocytosis with alendronate: evidence for a direct effect of bisphosphonate on histiocytes. Arthritis Rheum. 2003 Dec. 48(12):3538-41. [Medline].
Satoh M, Oyama N, Yamada H, Nakamura K, Kaneko F. Treatment trial of multicentric reticulohistiocytosis with a combination of predonisolone, methotrexate and alendronate. J Dermatol. 2008 Mar. 35(3):168-71. [Medline].
Sellam J, Deslandre CJ, Dubreuil F, Arfi S, Kahan A. Refractory multicentric reticulohistiocytosis treated by infliximab: two cases. Clin Exp Rheumatol. 2005 Jan-Feb. 23(1):97-9. [Medline].
Kalajian AH, Callen JP. Multicentric reticulohistiocytosis successfully treated with infliximab: an illustrative case and evaluation of cytokine expression supporting anti-tumor necrosis factor therapy. Arch Dermatol. 2008 Oct. 144(10):1360-6. [Medline].
Yeter KC, Arkfeld DG. Treatment of multicentric reticulohistiocytosis with adalimumab, minocycline, methotrexate. Int J Rheum Dis. 2013 Feb. 16(1):105-6. [Medline].
De Knop KJ, Aerts NE, Ebo DG, Van Offel JF, Stevens WJ, De Clerck LS. Multicentric reticulohistiocytosis associated arthritis responding to anti-TNF and methotrexate. Acta Clin Belg. 2011 Jan-Feb. 66(1):66-9. [Medline].
Cash JM, Tyree J, Recht M. Severe multicentric reticulohistiocytosis: disease stabilization achieved with methotrexate and hydroxychloroquine. J Rheumatol. 1997 Nov. 24(11):2250-3. [Medline].
Lonsdale-Eccles AA, Haworth AE, McCrae FC, Young-Min SA. Successful treatment of multicentric reticulohistiocytosis with leflunomide. Br J Dermatol. 2009 Aug. 161(2):470-2. [Medline].
Shannon SE, Schumacher HR, Self S, Brown AN. Multicentric reticulohistiocytosis responding to tumor necrosis factor-alpha inhibition in a renal transplant patient. J Rheumatol. 2005 Mar. 32(3):565-7. [Medline].
Broadwell AW, Calamia KT, Kransdorf MJ, Ginsburg WW. Healing of erosive disease in multicentric reticulohistiocytosis. J Rheumatol. 2010 Jun. 37(6):1366-7. [Medline].
Mavragani CP, Batziou K, Aroni K, Pikazis D, Manoussakis MN. Alleviation of polyarticular syndrome in multicentric reticulohistiocytosis with intravenous zoledronate. Ann Rheum Dis. 2005 Oct. 64(10):1521-2. [Medline]. [Full Text].
Pacheco-Tena C, Reyes-Cordero G, Ochoa-Albíztegui R, Ríos-Barrera V, González-Chávez SA. Treatment of multicentric reticulohistiocytosis with tocilizumab. J Clin Rheumatol. 2013 Aug. 19(5):272-6. [Medline].
Selmi C, Greenspan A, Huntley A, Gershwin ME. Multicentric reticulohistiocytosis: a critical review. Curr Rheumatol Rep. 2015 Jun. 17 (6):511. [Medline].
Lotti T, Santucci M, Casigliani R, Fabbri P, Bondi R, Panconesi E. Multicentric reticulohistiocytosis. Report of three cases with the evaluation of tissue proteinase activity. Am J Dermatopathol. 1988 Dec. 10(6):497-504. [Medline].
Gorman JD, Danning C, Schumacher HR, Klippel JH, Davis JC Jr. Multicentric reticulohistiocytosis: case report with immunohistochemical analysis and literature review. Arthritis Rheum. 2000 Apr. 43(4):930-8. [Medline].
Nakamura H, Yoshino S, Shiga H, Tanaka H, Katsumata S. A case of spontaneous femoral neck fracture associated with multicentric reticulohistiocytosis: oversecretion of interleukin-1beta, interleukin-6, and tumor necrosis factor alpha by affected synovial cells. Arthritis Rheum. 1997 Dec. 40(12):2266-70. [Medline].
Iwata H, Okumura Y, Seishima M, Aoyama Y. Overexpression of monocyte chemoattractant protein-1 in the overlying epidermis of multicentric reticulohistiocytosis lesions: a case report. Int J Dermatol. 2012 Apr. 51(4):492-4. [Medline].
Adamopoulos IE, Wordsworth PB, Edwards JR, Ferguson DJ, Athanasou NA. Osteoclast differentiation and bone resorption in multicentric reticulohistiocytosis. Hum Pathol. 2006. 37(9):1176–85.
Islam AD, Naguwa SM, Cheema GS, Hunter JC, Gershwin ME. Multicentric reticulohistiocytosis: A rare yet challenging disease. Clin Rev Allergy Immunol. 2013 Feb 17.
Valencia IC, Colsky A, Berman B. Multicentric reticulohistiocytosis associated with recurrent breast carcinoma. J Am Acad Dermatol. 1998 Nov. 39(5 Pt 2):864-6. [Medline].
Tan BH, Barry CI, Wick MR, et al. Multicentric reticulohistiocytosis and urologic carcinomas: a possible paraneoplastic association. J Cutan Pathol. 2011 Jan. 38(1):43-8. [Medline].
El-Haddad B, Hammoud D, Shaver T, Shahouri S. Malignancy-associated multicentric reticulohistiocytosis. Rheumatol Int. 2011 Sep. 31(9):1235-8. [Medline].
Kishikawa T, Miyashita T, Fujiwara E, Shimomura O, Yasuhi I, Niino D, et al. Multicentric reticulohistiocytosis associated with ovarian cancer. Mod Rheumatol. 2007. 17(5):422-5. [Medline].
Huang X, Zhang L, Zhang S. Multicentric reticulohistiocytosis with extra-mammillary Paget's disease: a case report. Eur J Med Res. 2013 Oct 29. 18(1):38. [Medline].
Takahashi M, Mizutani H, Nakamura Y, Shimizu M. A case of multicentric reticulohistiocytosis, systemic sclerosis and Sjögren syndrome. J Dermatol. 1997 Aug. 24(8):530-4. [Medline].
West KL, Sporn T, Puri PK. Multicentric reticulohistiocytosis: a unique case with pulmonary fibrosis. Arch Dermatol. 2012 Feb. 148(2):228-32. [Medline].
Webb-Detiege T, Sasken H, Kaur P. Infiltration of histiocytes and multinucleated giant cells in the myocardium of a patient with multicentric reticulohistiocytosis. J Clin Rheumatol. 2009 Feb. 15(1):25-6. [Medline].
Benucci M, Sulla A, Manfredi M. Cardiac engagement in multicentric reticulohistiocytosis: report of a case with fatal outcome and literature review. Intern Emerg Med. 2008 Jun. 3(2):165-8. [Medline].
Lambert CM, Nuki G. Multicentric reticulohistiocytosis with arthritis and cardiac infiltration: regression following treatment for underlying malignancy. Ann Rheum Dis. 1992 Jun. 51(6):815-7. [Medline]. [Full Text].
Fast A. Cardiopulmonary complications in multicentric reticulohistiocytosis. Report of a case. Arch Dermatol. 1976 Aug. 112(8):1139-41. [Medline].
Yang HJ, Ding YQ, Deng YJ. Multicentric reticulohistiocytosis with lungs and liver involved. Clin Exp Dermatol. 2009 Mar. 34(2):183-5. [Medline].
Taniguchi T, Asano Y, Okada A, Sugaya M, Sato S. Ultraviolet light-induced Köbner phenomenon contributes to the development of skin eruptions in multicentric reticulohistiocytosis. Acta Derm Venereol. 2011 Mar. 91(2):160-3. [Medline].
Fett N, Liu RH. Multicentric reticulohistiocytosis with dermatomyositis-like features: a more common disease presentation than previously thought. Dermatology. 2011. 222(2):102-8. [Medline].
Mun JH, Ko HC, Kim MB. Multicentric reticulohistiocytosis masquerading as dermatomyositis: similar and different features. J Dermatol. 2012 Jan. 39(1):104-7. [Medline].
Bennassar A, Mas A, Guilabert A, Julia M, Mascaro-Galy JM, Herrero C. Multicentric reticulohistiocytosis with elevated cytokine serum levels. J Dermatol. 2011 Sep. 38(9):905-10. [Medline].
Wat M, Sun GS, Rodriguez-Waitkus PM, Hsu S. Granuloma annulare mimicking multicentric reticulohistiocytosis. Dermatol Online J. 2013 Nov 15. 19(11):20400. [Medline].
Yamada T, Kurohori YN, Kashiwazaki S, Fujibayashi M, Ohkawa T. MRI of multicentric reticulohistiocytosis. J Comput Assist Tomogr. 1996 Sep-Oct. 20(5):838-40. [Medline].
Kamel H, Gibson G, Cassidy M. Case report: the CT demonstration of soft tissue involvement in multicentric reticulohistiocytosis. Clin Radiol. 1996 Jun. 51(6):440-1. [Medline].
Zhang B, Zhou H, Han J, Shi X, Zhang X. 18F-FDG PET/CT Findings in Multicentric Reticulohistiocytosis. Clin Nucl Med. 2015 Nov 13. [Medline].
Wade RG, Daivajna S, Chapman P, Murphy JG, Makkuni D. Hand surgery for Multicentric Reticulohistiocytosis: A new avenue of treatment and review of the literature. Int J Surg Case Rep. 2013. 4(8):744-7. [Medline]. [Full Text].
Motegi SI, Yonemoto Y, Yanagisawa S, Toki S, Uchiyama A, Yamada K, et al. Successful Treatment of Multicentric Reticulohistiocytosis with Adalimumab, Prednisolone and Methotrexate. Acta Derm Venereol. 2015 Jun 15. [Medline].