Multicentric Reticulohistiocytosis Treatment & Management

Updated: Dec 29, 2015
  • Author: Alisa N Femia, MD; Chief Editor: Herbert S Diamond, MD  more...
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Treatment

Approach Considerations

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.

Surgical care

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. [56]

In the case of internal malignancies, adequate tumor removal may result in the resolution of histiocytosis. [38]

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. [27]

Activity

Activity may be limited by the severity of MRH. Physical therapy may prevent deformities and relieve symptoms.

Consultations

The following consultations may be necessary:

  • Dermatologists
  • Rheumatologists
  • Oncologists or surgeons: May be needed if internal malignancy occurs

Monitoring

Patients with MRH should be monitored at regular intervals to track the activity of the disease and response to therapy.

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Pharmacologic 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, [5] 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 [14] 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. [26]

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