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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Multicentric reticulohistiocytosis


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Symptoms

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What are the symptoms of multicentric reticulohistiocytosis?

The main symptoms of multicentric reticulohistiocytosis are arthritis and red to purple skin nodules varying in size from 1 to 10 mm. The nodules can be found on any part of the body but tend to concentrate on the face and hands and decrease in number from head to toe.[1][2] The arthritis is most often symmetrical and polyarticular (affecting many joints). Unlike adult rheumatoid arthritis, it does not spare the joints closest to the fingertips. It can be severely destructive, and in one third of cases it progresses to arthritis multilans. Further history reveals that approximately one third of patients complain of symptoms such as fever, weight loss, and malaise; less often, pericarditis and myositis are present.[1][2]

The clinical presentation of multicentric reticulohistiocytosis is insidious in onset and begins with arthritic complaints in approximately two thirds of patients.  It is potentially one of the most rapidly destructive forms of arthritis. Joint involvement remits and relapses, gradually worsening into a debilitating and permanent arthritis multilans.  The severity of the damage has been reported to be related to the age of onset; therefore, the earlier one has symptoms, the more severe the symptoms tend to be.  Like the associated arthritis, skin lesions tend to wax and wane until the disease spontaneously resolves, but may leave permanent disfigurement.[2]

Last updated: 6/21/2013

References
  1. West SG. Chapter 297 -- Systemic Diseases in Which Arthritis is a Feature. Goldman: Cecil Textbook of Medicine, 23rd ed.. Philadelphia, PA: Saunders Elsevier; 2008;
  2. Hsiung et al.. Multicentric reticulohistiocytosis presenting with clinical features of dermatomyositis. Journal of the American Academy of Dermatology. 2003;


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