Revista Colombiana de Reumatología
Print version ISSN 0121-8123
VARGAS, Francisco et al. Multicentric reticulohistiocytosis. Rev.Colomb.Reumatol. [online]. 2007, vol.14, n.3, pp. 219-228. ISSN 0121-8123.
Multicentric reticulohistiocytosis (MRH) is a disease of unknown etiology that affects primarily women in the fourth decade of life. Articular involvement is characterized by the presence of symmetric synovitis of the upper extremities, particularly of the hands, and compromise of the skin with lesions of different morphology, more commonly nodules and papules localized mainly on the face and upper extremities. There can also be involvement of internal organs such as lung, heart, gastrointestinal tract and salivary glands. This disease has been linked to malignant neoplasms of different organs like breast, ovary, uterine cervix and lymphomas, leading some authors to consider this disease a manifestation of a paraneoplastic syndrome. Laboratory testing in these patients show abnormalities of the complete blood count, liver function tests as well as markers of autoimmunity. Histological studies confirm the diagnosis by demonstrating giant multinucleated histiocytic cells (mononuclear histiocytes-diammeter of 50 to 100 µm) with an eosinophilic cytoplasm and fine granules that give the cells a ground glass appearance. Multiple medications have been used to treat this disease including cyclophosphamide, azathioprine, methotrexate, chlorambucil and combined regimens. We document the case of a 51 year old caucasian female patient that was evaluated because of a 4 month history of subjective fever, weakness, fatigue, weight loss, inflammatory bilateral polyarthritis of the wrists, elbows and knees as well as the appearance of painful violaceous papules on the surface of the face, elbows, hands and flanks.
Keywords : multicentric reticulohistiocytosis; histiocitic diseases; paraneoplastic syndrome.