Multicentric Reticulohistiocytosis Clinical Presentation

  • Author: Jeffrey P Callen, MD; Chief Editor: Herbert S Diamond, MD   more...
 
Updated: Aug 16, 2011
 

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.
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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.
    • Some patients also have a xanthelasma.
    • The nodules grow slowly, and they rarely ulcerate.
    • Infiltrated plaques may resemble mucinosis.
    • Some patients appear to have a photodistribution of lesions simulating dermatomyositis.[5] Multiple erythematous nodules are present on the dMultiple erythematous nodules are present on the dorsal hands of this adolescent with an inflammatory arthropathy. Multiple erythematous to brown nodules on the fingMultiple erythematous to brown nodules on the fingers. Erythematous, poikilodermatous mamillated plaque oErythematous, poikilodermatous mamillated plaque on the anterior chest.
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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 (perhaps 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,[6] 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.
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Contributor Information and Disclosures
Author

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, and American College of Rheumatology

Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Electrical Optical Sciences Consulting fee Consulting; Celgene Honoraria Safety Monitoring Committee; GSK - Glaxo Smith Kline Consulting fee Consulting; TenXBioPharma Consulting fee Safety Monitoring Committee

Specialty Editor Board

Bryan L Martin, DO  Associate Dean for Graduate Medical Education, Designated Institutional Official, Associate Medical Director, Director, Allergy Immunology Program, Professor of Medicine and Pediatrics, Ohio State University College of Medicine

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.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

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

Rajalaxmi McKenna, MD, FACP  Southwest Medical Consultants, SC, Department of Medicine, 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  Adjunct Professor of Medicine, Division of Rheumatology, University of Pittsburgh 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: Merck Ownership interest Other; Smith Kline Ownership interest Other; Zimmer Ownership interest Other

References

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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 the ground glass cytoplasm and multinucleated giant cells.
 
 
 
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