eMedicine Specialties > Dermatology > Benign Neoplasms

Multicentric Reticulohistiocytosis: Differential Diagnoses & Workup

Author: Ronald P Rapini, MD, Professor and Chair, Department of Dermatology, Professor of Pathology, University of Texas Medical School, MD Anderson Cancer Center
Contributor Information and Disclosures

Updated: Mar 10, 2008

Differential Diagnoses

Leprosy

Other Problems to Be Considered

Rheumatoid nodules
Progressive nodular histiocytomas
Farber disease (lipogranulomatosis)
Gouty tophi
Osteoarthritis
Rheumatoid arthritis
Gout

Workup

Laboratory Studies

  • About one half of patients may have an elevated erythrocyte sedimentation rate or anemia.
  • Some patients have thyroid function abnormalities.
  • About 6% of patients have diabetes.
  • One third of patients have hypercholesterolemia, often associated with xanthelasma, but this seems to be unrelated to MRH.
  • Rheumatoid factor is characteristically negative, but it has been reported to be positive in at least 4 cases.
  • Occasionally, patients have had paraproteinemia, cold agglutinins, cryoglobulinemia, or hypergammaglobulinemia.
  • Synovial fluid findings are variable, and reports exist of elevated counts of neutrophils or mononuclear cells.

Imaging Studies

  • Routine radiographs of joints may be helpful. Changes are most commonly seen in the interphalangeal joints (PIP or DIP).
    • Bilaterally symmetric, sharply circumscribed erosions spread from the margins to the joint surfaces.
    • Separation of the bone ends often occurs, but no subchondral sclerosis and little or no periosteal reaction are seen.
    • Osteoporosis is mild or absent.
    • Erosions in osteoarthritis begin centrally rather than at the joint margins.
    • In psoriatic arthritis and Reiter disease, erosions are asymmetric and have poorly defined margins; the erosions are also associated with periosteal new bone formation.
  • MRI2 and CT scanning3 have been reported to be helpful, but they are not needed in most cases.
  • Gallium scans and bone scans have been used in the past; however, recommending them routinely is difficult because of their nonspecificity.

Histologic Findings

The papulonodules of the skin and the larynx consist of a diffuse infiltration of true histiocytes (macrophages) that tend to have abundant ground-glass (nonfoamy) cytoplasm. When the skin lesions are present, skin is the easiest site from which to obtain a biopsy specimen. Sometimes these histiocytes are found after blind biopsies of synovium in patients with unclassified arthritis, who lack the skin lesions.4 The characteristic histiocytes can be huge or bizarre, and they may be multinucleated. Varying numbers of lymphocytes or eosinophils may be present.

The cytoplasm of the histiocytes stains with the periodic acid-Schiff (PAS) stain. Although positive staining with Sudan black B and scarlet red indicates the presence of lipid within these cells, they are not usually foamy to the degree found in many other histiocytic disorders. The cells stain with the usual macrophage markers, such as lysozyme, CD68, MAC387, or human alveolar macrophage-56 (HAM-56). Staining for S-100, CD1a, CD34, factor XIIIa, or alpha-1-antitrypsin is typically negative.

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. Kishikawa T, Miyashita T, Fujiwara E, Shimomura O, Yasuhi I, Niino D, et al. Multicentric reticulohistiocytosis associated with ovarian cancer. Mod Rheumatol. Oct 2007;17(5):422-5. [Medline].

  2. Yamada T, Kurohori YN, Kashiwazaki S, Fujibayashi M, Ohkawa T. MRI of multicentric reticulohistiocytosis. J Comput Assist Tomogr. Sep-Oct 1996;20(5):838-40. [Medline].

  3. Kamel H, Gibson G, Cassidy M. Case report: the CT demonstration of soft tissue involvement in multicentric reticulohistiocytosis. Clin Radiol. Jun 1996;51(6):440-1. [Medline].

  4. 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). Feb 2006;45(2):192-5. [Medline].

  5. Cash JM, Tyree J, Recht M. Severe multicentric reticulohistiocytosis: disease stabilization achieved with methotrexate and hydroxychloroquine. J Rheumatol. Nov 1997;24(11):2250-3. [Medline].

  6. 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].

  7. 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].

  8. Shannon SE, Schumacher HR, Self S, Brown AN. Multicentric reticulohistiocytosis responding to tumor necrosis factor-alpha inhibition in a renal transplant patient. J Rheumatol. Mar 2005;32(3):565-7. [Medline].

  9. 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].

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

  11. Mavragani CP, Batziou K, Aroni K, Pikazis D, Manoussakis MN. Alleviation of polyarticular syndrome in multicentric reticulohistiocytosis with intravenous zoledronate. Ann Rheum Dis. Oct 2005;64(10):1521-2. [Medline].

  12. Barrow MV, Holubar K. Multicentric reticulohistiocytosis. A review of 33 patients. Medicine (Baltimore). Jul 1969;48(4):287-305. [Medline].

  13. Brackenridge A, Bashir T, Wheatley T. Multicentric reticulohistiocytosis and pregnancy. BJOG. May 2005;112(5):672-3. [Medline].

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

  15. Luz FB, Gaspar AP, Ramos-e-Silva M, Carvalho da Fonseca E, Villar EG, Cordovil Pires AR, et al. Immunohistochemical profile of multicentric reticulohistiocytosis. Skinmed. Mar-Apr 2005;4(2):71-7. [Medline].

  16. Mody GM, Cassim B. Rheumatologic manifestations of malignancy. Curr Opin Rheumatol. Jan 1997;9(1):75-9. [Medline].

  17. Rapini RP. Multicentric reticulohistiocytosis. Clin Dermatol. Jan-Mar 1993;11(1):107-11. [Medline].

  18. Tajirian AL, Malik MK, Robinson-Bostom L, Lally EV. Multicentric reticulohistiocytosis. Clin Dermatol. Nov-Dec 2006;24(6):486-92. [Medline].

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

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Further Reading

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Keywords

MRH, lipoid dermatoarthritis, lipoid rheumatism, giant cell reticulohistiocytosis, arthritis mutilans, arthritis

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Contributor Information and Disclosures

Author

Ronald P Rapini, MD, Professor and Chair, Department of Dermatology, Professor of Pathology, University of Texas Medical School, MD Anderson Cancer Center
Ronald P Rapini, MD is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Society for Investigative Dermatology, Southern Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Takeji Nishikawa, MD, Emeritus Professor, Department of Dermatology, Keio University School of Medicine; Director, Samoncho Dermatology Clinic; Managing Director, The Waksman Foundation of Japan Inc
Disclosure: Nothing to disclose.

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA
Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Rosalie Elenitsas, MD, Associate Professor of Dermatology, University of Pennsylvania School of Medicine; Director, Penn Cutaneous Pathology Services, Department of Dermatology, University of Pennsylvania Health System
Rosalie Elenitsas, MD is a member of the following medical societies: American Society of Dermatopathology
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System
William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology
Disclosure: elsevier Royalty Other; american college of physicians Honoraria Other

 
 
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