Multicentric Reticulohistiocytosis: a Rare Yet Challenging Disease
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Multicentric Reticulohistiocytosis: a Rare Yet Challenging Disease Arshia D. Islam & Stanley M. Naguwa & Gurtej S. Cheema & John C. Hunter & M. Eric Gershwin
# Springer Science+Business Media New York 2013
Definition and Historical Perspective
reticulohistiocytosis (MRH). In their review, they defined this disorder as follows: “Multicentric Reticulohistiocytosis is a systemic disease of unknown cause manifested by the gradual development of nodules in the skin, mucosa, subcutaneous tissues, synovia and at times periosteum and bone, resulting in destructive arthritis and disfigurement of the facies. On biopsy of a nodule, whether from skin, mucosa or synovia, multinucleated giant cells and histiocytes with a ground glass appearance of the cytoplasm secondary to lipid inclusions are invariably seen” [1]. Overall, this description of MRH still holds true today. It is not clear when MRH was first described. Some references date back to as early as 1897 when Targett described a case which is felt by some authorities to be the first case of MRH. In 1932, Caro and Senear described a patient that was noted to have reticulohistiocytic granuloma. However, the first well-described case of this disease with systemic involvement was actually documented in 1937 by Weber and Frudenthal [1]. Over the course of time, various terms have been used to describe this clinical entity, including giant cell histiocytosis, lipoid dermatoarthritis, giant cell reticulohistiocytosis, and reticulohistiocytic granuloma [2]. The actual term, multicentric reticulohistiocytosis, was first used by Goltz and Laymon in 1954 [3].
In 1969, Barrow and Holubar published the first comprehensive review of 33 patients with multicentric
Epidemiology
Abstract Multicentric reticulohistiocytosis (MRH) is a rare systemic inflammatory granulomatous disease that primarily manifests clinically with severe erosive arthritis and widespread papulonodular skin lesions but can involve multiple other organ systems. Despite the fact that this condition can become aggressive, debilitating as well as deforming with significant detrimental consequences, the etiology of this disease remains poorly understood. Moreover, the fact that MRH is such an uncommon disease has created an obstacle in the path of adequate clinical trials that are needed for better understanding of this phenomenon and for the development of treatment options for this patient population. In this review, we will attempt to discuss the epidemiology, pathophysiology, clinical features, associated conditions, differential diagnoses, diagnostic workup, and available treatments of MRH with the hope of creating a better understanding of this very challenging yet elusive disease process. Keywords Papulonodular cutaneous lesions . Symmetric erosive arthritis . Histiocytosis . Multinucleated giant cells . Ground glass eosinophilic cytoplasm
A. D. Islam : S. M. Naguwa : G. S. Cheema : M. E. Gershwin (*) Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis School of Medic
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