Approach Considerations

No therapy consistently improves multicentric reticulohistiocytosis (MRH). After an average course of 7-8 years, MRH often goes 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.

Depending on its severity, MRH may limit activity. Physical therapy may prevent deformities and relieve symptoms.

Consultations with dermatologists and rheumatologists may be necessary. If internal malignancy occurs, consult with oncologists or surgeons as needed.

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

Medical Care

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,^{[6, 7]} chlorambucil,^[6] and methotrexate,^{[8, 9, 7, 10]} may also affect the inflammatory response, as well as prevent further joint destruction and cause skin lesions to regress. Azathioprine^{[13, 14]} and cyclosporine^[15] are also reportedly effective in MRH.

Individual patients have reportedly responded to treatment with alendronate and other bisphosphonates.^{[16, 17]} 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.^{[11, 12, 18, 19, 20, 21]}

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.^[27]

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.^[61]

In the case of internal malignancies, adequate tumor removal may result in the resolution of histiocytosis.^[39]

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.^[28]

Medication

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Media Gallery

Multiple erythematous nodules are present on the dorsal hands of this adolescent with an inflammatory arthropathy.
Multiple erythematous to brown nodules on the fingers.
Erythematous, poikilodermatous, mamillated plaque on the anterior chest.
Histopathology of multicentric reticulohistiocytosis (MRH) skin lesions.
Histopathology of multicentric reticulohistiocytosis (MRH) skin lesions. Higher power demonstrating multinucleated giant cells with eosinophilic ground-glass cytoplasm.
Erythematous to brown papules overlying the right dorsal hand and wrist and erythematous to violaceous patches over the right dorsal hand and fingers. The cutaneous changes are in a photodistributed pattern and mimic the changes of dermatomyositis.
Erythematous to brown papules and erythematous to violaceous patches overlying the right dorsal hand and fingers, with a crusted erosion overlying the fourth metacarpophalangeal joint. These cutaneous changes can be easily confused with dermatomyositis.
Nodules on a hand with deformed joints from arthritis due to multicentric reticulohistiocytosis.
Swollen elbow and nodules on the forearm.
Cobblestone papules on the eyelid and papules on the forehead.
Low-power view of a biopsy sample of large histiocytes and multinucleated giant cells in the dermis.
High-power view of large histiocytes in the dermis.
Brown immunoperoxidase staining for lysozyme in histiocytes.

of 13

Author

Alisa N Femia, MD Assistant Professor, Ronald O Perelman Department of Dermatology, New York University Medical Center

Alisa N Femia, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Society for Investigative Dermatology, Medical Dermatology Society, Rheumatologic Dermatology Society

Disclosure: Nothing to disclose.

Coauthor(s)

Ruth Ann Vleugels, MD, MPH Assistant Professor of Dermatology, Harvard Medical School; Associate Physician, Department of Dermatology, Brigham and Women's Hospital; Associate Physician, Department of Immunology and Allergy, Children's Hospital Boston

Ruth Ann Vleugels, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Rheumatology, American Medical Association, Society for Investigative Dermatology, Medical Dermatology Society, Dermatology Foundation

Disclosure: Nothing to disclose.

Jeffrey P Callen, MD Professor of Medicine (Dermatology), 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, American College of Rheumatology

Disclosure: Received honoraria from UpToDate for author/editor; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for i inherited these trust accounts; I serve on a safety monitoring committee for Principia Biopharma for: Allergen; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble; Amgen; Gilead.

Ronald P Rapini, MD Professor and Chair, Department of Dermatology, The University of Texas MD Anderson Cancer Center; Distinguished Chernosky Professor and Chair of Dermatology, Professor of Pathology, University of Texas McGovern Medical School at Houston

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, American Society of Dermatopathology, Association of Professors of Dermatology, Society for Investigative Dermatology, Texas Dermatological Society

Disclosure: Book royalties from Elsevier publishers.

Chief Editor

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; 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, Phi Beta Kappa

Disclosure: Nothing to disclose.

Acknowledgements

Marcel E Conrad, MD Distinguished Professor of Medicine (Retired), University of South Alabama College of Medicine

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

Rosalie Elenitsas, MD Herman Beerman 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 Academy of Dermatology and American Society of Dermatopathology

Disclosure: Lippincott Williams Wilkins Royalty Textbook editor

Dirk M Elston, MD Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology