The authors present part I of a review of multicentric reticulohistiocytosis, a rare systemic proliferative disease of histiocytes of unknown cause. It is clinically characterized by cutaneous and mucosal nodules and by osteoarticular lesions. The disease occurs in outbreaks that progress in severity, with spontaneous regression, but usually leaving incapacitating arthritis and disfiguring facial lesions. The authors discuss the historical, epidemiologic, and clinical aspects of this disease.
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