Clinical features of multicentric reticulohistiocytosis

Objective To study the clinical profile of patients with multicentric reticulohistiocytosis (MRH). Methods The clinical manifestations, image and histologic characteristics of ten patients with MRH were collected, and the relevant literatures were reviewed. Results Seven cases were women. The median (quartile) age was 46.5 (44.5-55.3) years. Clinical symptoms included papulonodular lesions on the skin (ten cases), erosive polyarthritis (ten cases), myositis (four cases), lung nodules (three cases), larynx nodules (one case) and serositis (one case). Biopsy demonstrated infiltration of multinucleated giant cells with CD68 positive and "glassy" cytoplasm. All of the ten patients were treated with corticosteroids and disease -modifying antirheumatic drugs, skin lesions of seven patients were ameliorated and arthritis was improved in three patients. Conclusion MRH is a systemic disease that has characteristic clinical manifestations and unique histological findings. Deep understanding of this disease can avoid misdiagnose. Further studies are needed to explore effective treatment. Key words: Histiocytosis; Arthritis; Papules nodules