Multicentric reticulohistiocytosis is a rare disease affecting skin and joints primarily and rarely other organs. We present a case report of this disease and an extensive review of the literature. We reviewed the data between 1991 and 2014 and extracted 52 individual cases. Only articles in English were chosen after checking for relevance. The articles were studies and data was extracted into excel spread sheets and later used to compute such variables like frequency, mean and percentage of distribution of various clinical manifestations. The treatments used in these articles were critically analyzed and graded for their relative efficacy for skin and joint manifestations. The grades were 0 = worse, 1 = no benefit/condition remained same, 2 = improvement without resolution, and 3 = resolution. This article also reports the demographic, clinical, laboratory and pathological data from the reviewed articles. Authors attempted to discuss the findings of this review in depth to help manage this condition and proposed a treatment algorithm to help clinicians approach this rare and challenging disease.
Keywords: Arthritis; Autoimmune disease; Immunosuppressive medications; Skin disease.