Dermatologic Manifestations of Multicentric Reticulohistiocytosis Clinical Presentation

  • Author: Ronald P Rapini, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Oct 11, 2010
 

History

  • About one half of patients initially develop polyarthritis. One fourth of patients first develop the skin papules and the nodules. Another one fourth of patients develop skin and joint manifestations at the same time.
  • Skin lesions are usually asymptomatic.
  • One third of patients complain of pruritus.
  • Arthritis may wax and wane, but it can rapidly become severe.
  • About one third of patients have constitutional symptoms, such as weakness, weight loss, and fever.
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Physical

  • Skin lesions are skin colored to reddish brown.
  • Lesions vary from papules that are 1-2 mm in diameter to nodules that are several centimeters in diameter.
  • Papules and lesions may be isolated from one another, or they may be clustered, sometimes giving a cobblestone appearance (see the image below). Cobblestone papules on the eyelid and papules on tCobblestone papules on the eyelid and papules on the forehead.
  • The Koebner phenomenon has been reported, wherein trauma to the skin gives rise to new lesions.
  • Skin lesions more commonly occur on the upper half of the body, but other areas can be involved.
    • Face and hands (90%)
    • Ears (76%)
    • Forearms (55%) as shown belowSwollen elbow and nodules on the forearm. Swollen elbow and nodules on the forearm.
    • Elbows (40%) as depicted above
    • Scalp (40%)
    • Mucosal surfaces, such as the lips, the tongue, the gingiva, and the nasal septum (50%)
  • Several miscellaneous nail changes have been described, but most are due to adjacent arthritis.
  • About one third of patients have been reported to have xanthelasma, but whether or not this is related to MRH is unclear.
  • MRH is a polyarthritis involving a wide variety of joints.
    • Hands (76%)
    • Knees (73%)
    • Shoulders (64%)
    • Wrists (64%)
    • Hips (61%)
    • Ankles (58%)
    • Elbows (58%)
    • Feet (58%)
    • Spine (52%)
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Causes

Most cases of MRH are of unknown cause, but, in about 28% of cases, the disease appears to be caused by a paraneoplastic disorder related to an underlying malignancy. MRH precedes the development of cancer in 73% of cases. Whether the malignancy is truly related to MRH is debated for several reasons.

  • No consistent type of neoplasm is associated with MRH. Most of the reported specific cancer types are reported less than 5 times each in the literature.
  • Because MRH is rare, a reporting bias exists in the literature toward reporting those cases with underlying malignancy, especially previously unreported malignancies. Some of these associations may be a coincidence.
  • The activity of the arthritis and the skin lesions of MRH may or may not be correlated with the eradication of the cancer, unlike some paraneoplastic disorders where removal of the malignancy may produce improvement in the paraneoplastic findings.
  • Some patients with MRH have been extensively studied or an autopsy has been performed with no evidence of cancer.
  • MRH has been reported with cancer of the breast (scirrhous, intraductal, unspecified types), cervix, colon (adenocarcinoma), stomach (adenocarcinoma), lung (bronchogenic carcinoma, mesothelioma of pleura), bronchus, larynx, ovary (medullary carcinoma, adenocarcinoma),[2] lymphoma, leukemia, sarcoma (omentum, axilla), and melanoma. MRH has also been reported with cancers of unknown primary.
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Contributor Information and Disclosures
Author

Ronald P Rapini, MD  Josey Professor and Chair, Department of Dermatology, Professor of Pathology, University of Texas Medical School at Houston and 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, and Texas Medical Association

Disclosure: Elsevier publishers Royalty Independent contractor

Specialty Editor Board

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.

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.

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; DLA Piper Consulting fee Consulting

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

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

Disclosure: Nothing to disclose.

References

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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 the histologic features of a biopsy sample of large histiocytes and multinucleated giant cells in the dermis.
High-power view of the histologic features of large histiocytes in the dermis.
Brown immunoperoxidase staining for lysozyme in histiocytes.
 
 
 
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