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Genetic and Rare Diseases Information Center (GARD)

Multicentric reticulohistiocytosis

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What are the signs and 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

The Human Phenotype Ontology provides the following list of signs and symptoms for Multicentric reticulohistiocytosis. If the information is available, the table below includes how often the symptom is seen in people with this condition. You can use the MedlinePlus Medical Dictionary to look up the definitions for these medical terms.

Signs and Symptoms Approximate number of patients (when available)
Abnormality of the oral cavity 90%
Abnormality of the skin 90%
Arthritis 90%
Abnormality of temperature regulation 7.5%
Decreased body weight 7.5%
Muscle weakness 7.5%

Last updated: 7/1/2016

The Human Phenotype Ontology (HPO) has collected information on how often a sign or symptom occurs in a condition. Much of this information comes from Orphanet, a European rare disease database. The frequency of a sign or symptom is usually listed as a rough estimate of the percentage of patients who have that feature.

The frequency may also be listed as a fraction. The first number of the fraction is how many people had the symptom, and the second number is the total number of people who were examined in one study. For example, a frequency of 25/25 means that in a study of 25 people all patients were found to have that symptom. Because these frequencies are based on a specific study, the fractions may be different if another group of patients are examined.

Sometimes, no information on frequency is available. In these cases, the sign or symptom may be rare or common.

  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;

See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.