Systemic lupus erythematosus with multicentric reticulohistiocytosis: A case report

BACKGROUND Multicentric reticulohistiocytosis(MRH)/systemic lupus erythematosus(SLE) overlap syndrome is an uncommon disease in the clinic and is diagnosed through characteristic clinical manifestations, histopathology, and immunopathology. Here, we report the case of a 30-year-old woman with SLE who developed MRH.CASE SUMMARY A 30-year-old woman with a history of polyarthritis for the past 12 years had multiple skin nodules on her body for 10 years, including the sacrococcygeal area, dorsum of the hands, interphalangeal joint of the feet and sternoclavicular joint. The histopathology of a biopsy of the distal interphalangeal joint of the hands revealed granulomatous inflammation, fibrous hyperplasia with ground-glass degeneration, inflammatory cell exudation and focal necrosis. The immunohistochemical stains showed positive staining for CD68 and negative staining for S100 and acid-fast staining. The patient was diagnosed with SLE with MRH. Her symptoms were improved after a combined treatment of prednisone, hydroxychloroquine and cyclophosphamide.CONCLUSION MRH/SLE overlap syndrome is difficult to diagnose and treat. Cyclophosphamide may be an alternative choice for the treatment of MRH.

Multicentric reticulohistiocytosis with prominent skin lesions and arthritis:A case report

BACKGROUND Multicentric reticulohistiocytosis(MRH)is a rare non-Langerhans histiocytosis of unknown etiology characterized by papulonodular skin lesions and progressive,erosive arthritis.To date,there have been approximately 300 cases of MRH reported worldwide.The majority of patients are Caucasian from western countries,and Asian patients are rare.Here,we report a case of MRH in a Chinese patient.CASE SUMMARY A 38-year-old male was admitted to the hospital with a rash that had persisted for over 2 years and bilateral knee pain for over 1 year.The patient’s symptoms had previously been misdiagnosed as eczema when there were only skin symptoms and was finally diagnosed as MRH after a skin biopsy of the left upper back.The patient was treated with glucocorticoids combined with an immunosuppressive regimen.While the skin lesions on both arms,abdomen,and back subsided,the skin lesions on the rest of the body did not increase.The interphalangeal joints of both thumbs and bilateral knee joints remained swollen and painful.CONCLUSION The case will help clinicians better identify and treat this disease in the absence of epidemiological studies or randomized controlled data.

A case of Sjogren's syndrome evolving into multicentric reticulohistiocytosis

Introduction:Multicentric 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 Description:A 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 2mm in size noted at the base of her nails.A 4mm 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.Conclusion:Our 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.