Abstract
Multicentric reticulohistiocytosis (MRH) is an uncommon systemic disease of unknown aetiology, which is characterized by the presence of papulonodular lesions, and is caused by the proliferation of histiocytes associated with arthritis. There are only just 200 cases described in the scientific literature. It can behave like a paraneoplasia and may be malignant in a quarter of the patients. We would like to add our patient to those described in the literature. Our patient was a 68 year-old male stonemason who was diagnosed of multicentric reticulohistiocytosis and was previously unsuccessfully treated with classical immunosupressants. The patient did, nonetheless, develop favourably with etanercept, with remission of the clinical and articular lesions. In our opinion we feel that new treatment hopes will emerge for the treatment of inflammatory diseases where tumoral necrosis factor (TNF)-alpha and other cytokine production by monocytes and macrophages will play an important role.
Highlights
Multicentric reticulohistiocytosis (MRH) is an uncommon systemic disease of unknown aetiology, which is characterized by the presence of papulonodular lesions, and is caused by the proliferation of histiocytes associated with arthritis
With the arrival of biological therapies, new treatment hopes for inflammatory diseases in which tumoral necrosis factor (TNF)-alpha production and other cytokines by monocytes and macrophages play an important role, will emerge
TNFalpha is found in the synovial fluid of patients suffering from rheumatoid arthritis and in severe forms of inflammatory arthritis, as well as in patients with MRH
Summary
Multicentric reticulohistiocytosis (MRH) is an uncommon systemic disease of unknown aetiology, which is characterized by the presence of papulonodular lesions, and is caused by the proliferation of histiocytes associated with arthritis. The term MRH was first used by Goltz and Laymon in 1954 [1] to distinguish it from cutaneous solitary reticulohistiocytosis [2], for describing patients showing destructive polyarthropathy and multiple cutaneous nodules. MRH is an uncommon condition with only 200 cases being described in the literature. It can behave like a paraneoplasia and can be malignant in a quarter of the patients. The case is of a 68-year-old male patient with multiple nodular lesions, which gradually appeared over a 4 year period, and which were accompanied by pruritus in the hands, arms and face. On the face we observed a nodule on the nasal pyramid (Fig. 1) and other smaller nodules in the nasal mucous membrane. Thoracic CAT revealed reticonodular infiltrate with a predomination of mean and upper fields, perilymphatic millimetric nodules, which caused retr-
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