A case of multicentric reticulohistiocytosis / 대한내과학회지

ABSTRACT

Multicentric reticulohistiocytosis (MRH) is a rare systemic disease, characterized by severe rapidly destructive polyarthritis and erythematous papulonodular skin lesions. This disorder involves primarily skin and joints, but it has been reported to affect nearly every organ system. Histological analyses of skin, mucosa and synovia reveal that multinucleated giant cells and proliferating histiocytes have smooth eosinophilic ground-glass cytoplasm. We report a case of a 46-year-old woman who was presented with multiple arthritis and erythematous patch on the V-neck and hard nodules on both hands, elbows, great toes, and auricles. About 25% of the reported patients with MRH have been associated with cancer, but MRH is not considered to be a paraneoplastic disorder. She has not been found any other malignant disease. She has been treated with NSAIDs, corticosteroid, alendronate, hydroxychloroquine, and low-dose methotrexate. Cyclosporin A was added to achieve complete remission later on. After 6 months, she was more improved in symptoms of multiple arthritis and skin nodules.