Multicentric reticulohistiocytosis in a Taiwanese woman with Sjögren syndrome

• Main Authors: Lan-hsin Cheng, Ying-Yi Chiang
• Format: Article
• Language: English
• Published: Wolters Kluwer Medknow Publications 2016-03-01
• Series: Dermatologica Sinica
• Subjects: Multicentric reticulohistiocytosis; non-langerhans cell histiocytosis; erosive polyarthropathy; dermatomyositis; Sjögren syndrome
• Online Access: http://www.sciencedirect.com/science/article/pii/S1027811715000993

Multicentric reticulohistiocytosis is a rare, non-Langerhans cell histiocytosis characterized by specific skin lesions and destructive arthritis. Clinically, it generally presents with multiple reddish-brown papulonodules over the hands, face, and trunk. The associated destructive arthropathy tends to be severe, with 50% of patients developing arthritis mutilans. Approximately 25–30% of patients present with neoplasia. Coexisting autoimmune diseases are also commonly reported. Here we report the case of a 59-year-old Taiwanese woman with underlying Sjögren syndrome who presented with the typical skin manifestations of multicentric reticulohistiocytosis and early interphalangeal joint involvement. The patient later showed partial response to methotrexate treatment.