A case of Sjogren's syndrome evolving into multicentric reticulohistiocytosis

IntroductionMulticentric reticulohistiocytosis (MRH) is a rare disease that is known to affect the skin and joints, primarily. It is considered a rare form of non-Langerhans cell histiocytosis (Group C) that can cause destructive inflammatory arthritis involving both the small and large joints. Cutaneous eruptions of periungual, "coral beads" and nodules appearing over the distal fingers are considered pathognomonic clues for identifying this disorder. Histology evaluation of the cutaneous papules typically shows infiltrative histiocytes and multinucleated giant cells. Although no well-established therapies exist to date, a variety of immunosuppressants have been used with varying degrees of success. Case DescriptionA 53-year-old Caucasian female patient with a family history of rheumatoid arthritis and a personal history of Sjogren's syndrome presented to the rheumatology clinic complaining of pain in her bilateral hands and fingers. There were several small, papulo-nodular lesions ranging from 1 to 2 mm in size noted at the base of her nails. A 4 mm punch biopsy of one of the papules from the neck showed dermal infiltration of eosinophilic mononucleated and multinucleated giant cells with "ground glass" appearing cytoplasm consistent with MRH. X-ray of her hands showed periarticular demineralization and erosions surrounding several bilateral proximal and distal interphalangeal joints, and thus tofacitinib in addition to methotrexate, hydroxychloroquine, and dexamethasone (dosed weekly) was started to help control any further articular damage. ConclusionOur aim is to further support the relation of MRH with autoimmune diseases, including Sjogren's syndrome. Autoimmune diseases have been reported in association with MRH, although a clear association has yet to be made.