eMedicine Specialties > Rheumatology > Miscellaneous Inflammatory Arthritis

Multicentric Reticulohistiocytosis

Author: Jeffrey P Callen, MD, Professor of Medicine, Chief, Division of Dermatology, University of Louisville School of Medicine
Contributor Information and Disclosures

Updated: Jul 17, 2009

Introduction

Background

In 1954, Goltz and Laymon coined the term multicentric reticulohistiocytosis (MRH) to describe patients with destructive arthritis and multiple cutaneous nodules that histologically manifest as an infiltrate of histiocytic multinucleated giant cells with eosinophilic ground-glass cytoplasm.1 This is a rare condition; fewer than 200 cases have been reported in the world literature. Various diseases have been associated with MRH, including malignancy.2

Pathophysiology

The pathogenesis of this condition is unknown but probably has an immunologic basis. Some studies have demonstrated increased levels of tumor necrosis factor (TNF)–alpha in the blood and in the tissue.3,4

Frequency

United States

MRH is a very rare condition, and no studies have detailed the prevalence in the general population.

International

MRH is a rare condition.

Mortality/Morbidity

MRH is a debilitating process but rarely causes death unless the patient has an associated malignancy.

Race

MRH has been reported primarily in whites.

Sex

Women are affected more commonly than men, with a female-to-male ratio of 2:1.

Age

MRH may occur at any age, but it has been reported primarily in middle-aged adults, with a mean age of 43 years.

Clinical

History

  • The primary manifestations of multicentric reticulohistiocytosis (MRH) are joint and skin diseases.
    • Inflammatory joint disease is a frequent presenting symptom, and it is the sole symptom in 45% of patients.
    • Skin disease is common, and it is the presenting manifestation in 30% of patients.

Physical

  • The joint disease usually manifests as destructive and deforming symmetrical polyarthritis with a predilection for the distal interphalangeal joints.
  • The classic skin lesions are multiple nodules that have a predilection for the hands, particularly the base of the nails, but they may occur on any body surface.
    • The lesions are usually nontender.
    • The nodules may be skin colored, red, or yellowish.
    • They vary in size from 1-10 mm and can coalesce to form plaques with a cobblestone surface.
    • One third of patients also have a xanthelasma.
    • The nodules grow slowly, and they rarely ulcerate.
    • Infiltrated plaques may resemble mucinosis.

      Multiple erythematous nodules are present on the ...

      Multiple erythematous nodules are present on the dorsal hands of this adolescent with an inflammatory arthropathy.

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      Multiple erythematous nodules are present on the ...

      Multiple erythematous nodules are present on the dorsal hands of this adolescent with an inflammatory arthropathy.



      Multiple erythematous to brown nodules on the fin...

      Multiple erythematous to brown nodules on the fingers.

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      Multiple erythematous to brown nodules on the fin...

      Multiple erythematous to brown nodules on the fingers.



      Erythematous, poikilodermatous mamillated plaque ...

      Erythematous, poikilodermatous mamillated plaque on the anterior chest.

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      Erythematous, poikilodermatous mamillated plaque ...

      Erythematous, poikilodermatous mamillated plaque on the anterior chest.

Causes

The cause of MRH is unknown, but various associated diseases have been reported in patients with MRH.

  • Malignancy has been reported in multiple patients (as many as 25%).
    • No specific site or type of malignancy has been reported.
    • Those reported include melanoma, sarcoma, leukemia, lymphoma, and carcinomas (usually adenocarcinoma) of the breast,5 colon, bronchus, cervix, stomach, and ovaries.
  • Various endocrinopathies have been reported, including diabetes mellitus and hypothyroidism.
  • Other associated conditions include rheumatoid arthritis, systemic lupus erythematosus, Sjögren syndrome, primary biliary cirrhosis, and pregnancy.

More on Multicentric Reticulohistiocytosis

Overview: Multicentric Reticulohistiocytosis
Differential Diagnoses & Workup: Multicentric Reticulohistiocytosis
Treatment & Medication: Multicentric Reticulohistiocytosis
Follow-up: Multicentric Reticulohistiocytosis
Multimedia: Multicentric Reticulohistiocytosis
References
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References

  1. Goltz RW, Laymon CW. Multicentric reticulohistiocytosis of the skin and synovia; reticulohistiocytoma or ganglioneuroma. AMA Arch Derm Syphilol. Jun 1954;69(6):717-31. [Medline].

  2. Nunnink JC, Krusinski PA, Yates JW. Multicentric reticulohistiocytosis and cancer: a case report and review of the literature. Med Pediatr Oncol. 1985;13(5):273-9. [Medline].

  3. Kovach BT, Calamia KT, Walsh JS, Ginsburg WW. Treatment of multicentric reticulohistiocytosis with etanercept. Arch Dermatol. Aug 2004;140(8):919-21. [Medline].

  4. Lovelace K, Loyd A, Adelson D, Crowson N, Taylor JR, Cornelison R. Etanercept and the treatment of multicentric reticulohistiocytosis. Arch Dermatol. Sep 2005;141(9):1167-8. [Medline].

  5. Valencia IC, Colsky A, Berman B. Multicentric reticulohistiocytosis associated with recurrent breast carcinoma. J Am Acad Dermatol. Nov 1998;39(5 Pt 2):864-6. [Medline].

  6. Ginsburg WW, O'Duffy JD, Morris JL, Huston KA. Multicentric reticulohistiocytosis: response to alkylating agents in six patients. Ann Intern Med. Sep 1 1989;111(5):384-8. [Medline].

  7. 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. Jan 1996;39(1):171-4. [Medline].

  8. Franck N, Amor B, Ayral X, et al. Multicentric reticulohistiocytosis and methotrexate. J Am Acad Dermatol. Sep 1995;33(3):524-5. [Medline].

  9. Gourmelen O, Le Loet X, Fortier-Beaulieu M, et al. Methotrexate treatment of multicentric reticulohistiocytosis. J Rheumatol. Apr 1991;18(4):627-8. [Medline].

  10. Rentsch JL, Martin EM, Harrison LC, Wicks IP. Prolonged response of multicentric reticulohistiocytosis to low dose methotrexate. J Rheumatol. May 1998;25(5):1012-5. [Medline].

  11. 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. Dec 2003;48(12):3538-41. [Medline].

  12. Sellam J, Deslandre CJ, Dubreuil F, et al. Refractory multicentric reticulohistiocytosis treated by infliximab: two cases. Clin Exp Rheumatol. Jan-Feb 2005;23(1):97-9. [Medline].

  13. 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. Oct 2008;144(10):1360-6. [Medline].

  14. Barrow MV, Holubar K. Multicentric reticulohistiocytosis. A review of 33 patients. Medicine (Baltimore). Jul 1969;DA - 19690930(4):287-305. [Medline].

  15. Blanco JJ, Hernandez FJ, Cerezo JG, et al. Multicentric reticulohistiocytosis. The long course of a rare disease. Scand J Rheumatol. 2002;31(2):107-9. [Medline].

  16. Callen JP. Miscellaneous disorders that commonly affect both skin and joints. In: Provost TT, ed. Cutaneous Manifestations of Rheumatic Diseases. Lippincott Williams & Wilkins; 1996:278-9.

  17. Candell Chalom E, Elenitsas R, Rosenstein ED, Kramer N. A case of multicentric reticulohistiocytosis in a 6-year-old child. J Rheumatol. Apr 1998;25(4):794-7. [Medline].

  18. Cox NH, West NC, Popple AW. Multicentric reticulohistiocytosis associated with idiopathic myelofibrosis. Br J Dermatol. Dec 2001;145(6):1033-4. [Medline].

  19. Hiramanek N, Kossard S, Barnetson RS. Multicentric reticulohistiocytosis presenting with a rash and arthralgia. Australas J Dermatol. May 2002;43(2):136-9. [Medline].

  20. Hsiung SH, Chan EF, Elenitsas R, et al. Multicentric reticulohistiocytosis presenting with clinical features of dermatomyositis. J Am Acad Dermatol. Feb 2003;48(2 Suppl):S11-4. [Medline].

  21. Lesher JL Jr, Allen BS. Multicentric reticulohistiocytosis. J Am Acad Dermatol. Oct 1984;DA - 19841212(4 Pt 2):713-23. [Medline].

  22. Orkin M, Goltz RW, Good RA, et al. A study of multicentric reticulohistiocytosis. Arch Dermatol. May 1964;89:640-54. [Medline].

  23. Outland JD, Keiran SJ, Schikler KN, Callen JP. Multicentric reticulohistiocytosis in a 14-year-old girl. Pediatr Dermatol. Nov-Dec 2002;19(6):527-31. [Medline].

  24. Peters MS. Histiocytic and Langerhans cell reactions. In: Farmer ER, Hood AF, eds. Pathology of the Skin. 1990:263-5.

  25. Raphael SA, Cowdery SL, Faerber EN, et al. Multicentric reticulohistiocytosis in a child. J Pediatr. Feb 1989;114(2):266-9. [Medline].

  26. Santilli D, Lo Monaco A, Cavazzini PL, Trotta F. Multicentric reticulohistiocytosis: a rare cause of erosive arthropathy of the distal interphalangeal finger joints. Ann Rheum Dis. Jun 2002;61(6):485-7. [Medline].

  27. Trotta F, Castellino G, Lo Monaco A. Multicentric reticulohistiocytosis. Best Pract Res Clin Rheumatol. Oct 2004;18(5):759-72. [Medline].

  28. Weber FP, Freudenthal W. Nodular non-diabetic cutaneous xanthomatosis with hypercholesterolemia and atypical histologic features. Proc R Soc Med. 1937;30:522-6.

  29. Yee KC, Bowker CM, Tan CY, Palmer RG. Cardiac and systemic complications in multicentric reticulohistiocytosis. Clin Exp Dermatol. Nov 1993;18(6):555-8. [Medline].

  30. Zvaifler NJ. Uncommon arthropathies. In: Klippel JH, Dieppe PA, eds. Rheumatology. 2nd ed. 1998:4-5.

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Further Reading

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Keywords

multicentric reticulohistiocytosis, MRH, lipoid dermatoarthritis, destructive arthritis, multiple cutaneous nodules, histiocytic multinucleated giant cells, eosinophilic ground-glass cytoplasm, inflammatory arthropathy, inflammatory joint disease, symmetrical polyarthritis

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Contributor Information and Disclosures

Author

Jeffrey P Callen, MD, Professor of Medicine, Chief, Division of Dermatology, University of Louisville School of Medicine
Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and American College of Rheumatology
Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Electrical Optical Sciences Honoraria Consulting; Centocor Honoraria Consulting; Genetech Honoraria Consulting; Celgene Honoraria Consulting

Medical Editor

Bryan L Martin, DO, Chief, Allergy Immunology Department, Walter Reed Army Medical Center; Associate Professor of Medicine and Pediatrics, Uniformed Services University of the Health Sciences; United States Army Consultant in Allergy Immunology and Immunizations
Bryan L Martin, DO is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American College of Allergy, Asthma and Immunology, American College of Osteopathic Internists, American College of Physicians, American Medical Association, and American Osteopathic Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Marcel E Conrad, MD, (Retired) Distinguished Professor of Medicine, University of South Alabama
Marcel E Conrad, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for the Advancement of Science, American Association of Blood Banks, American Chemical Society, American College of Physicians, American Physiological Society, American Society for Clinical Investigation, American Society of Hematology, Association of American Physicians, Association of Military Surgeons of the US, International Society of Hematology, Society for Experimental Biology and Medicine, and Southwest Oncology Group
Disclosure: No financial interests None None

CME Editor

Rajalaxmi McKenna, MD, FACP, Consulting Staff, Department of Medicine, Southwest Medical Consultants, SC, Good Samaritan Hospital, Advocate Health Systems
Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis
Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD, Professor of Medicine, Temple University School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital
Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa
Disclosure: medifocus Honoraria Review panel membership; health dialogs Honoraria Consulting; West Penn Allegheny Health System None Board membership

 
 
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