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Multicentric reticulohistiocytosis.
Ani L Tajirian1, Mohsin K Malik, Leslie Robinson-Bostom
1Department of Dermatology, Brown Medical School, Rhode Island Hospital, Providence, RI 02903, USA. ani_tajirian@brown.edu
Clinics in Dermatology
|November 23, 2006
Summary
Multicentric reticulohistiocytosis is a rare granulomatous disease causing skin nodules and destructive arthritis. Diagnosis relies on identifying characteristic histiocytes and giant cells in affected tissues.
Area of Science:
- Rheumatology
- Dermatology
- Pathology
Background:
- Multicentric reticulohistiocytosis (MRH) is a rare granulomatous disease of unknown cause.
- It presents with characteristic skin nodules and destructive arthritis, affecting approximately 50% of patients.
- The association with malignancy is noted, but its paraneoplastic nature remains unconfirmed.
Purpose of the Study:
- To summarize the key features of multicentric reticulohistiocytosis.
- To highlight diagnostic criteria and clinical manifestations.
- To discuss the potential link with malignancy.
Main Methods:
- Review of existing literature on multicentric reticulohistiocytosis.
- Analysis of histopathological findings in cutaneous nodules and synovial membranes.
- Correlation of clinical presentation with diagnostic markers.
Main Results:
- Multicentric reticulohistiocytosis is characterized by cutaneous nodules and severe, disabling arthritis.
- Histopathology reveals characteristic oncocytic ("ground-glass") histiocytes and multinucleated giant cells.
- A significant association with underlying malignancy exists, though causality is not established.
Conclusions:
- Multicentric reticulohistiocytosis requires histopathological confirmation for diagnosis.
- The disease can lead to significant disfigurement and disability.
- Further research is needed to elucidate the paraneoplastic potential and etiology.
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