Multicentric reticulohistiocytosis without arthritis successfully treated with systemic corticosteroids - A case report and literature review

A 46-year-old male presented with a 2-month history of multiple red papules on the cheeks and extremities without mucosal involvement or arthritis. There were only some lesions on the dorsal hands without periungual involvement. Biopsy of a skin lesion showed a dermal infiltrate consisting of mononuclear and multinucleated histiocytes with granular "ground glass"-like cytoplasm. These histiocytes were CD68-positive, and S-100 protein and CD30 negative in immunohistochemical studies. The diagnosis of multicentric reticulohistiocytosis without arthritis was made. Laboratory data were within normal limits except mild hypercholesterolemia and hypertriglyceridemia. A survey for internal malignancy showed negative results. The skin lesions responded to prednisolone 0.5 mg/kg daily. The dose was tapered gradually; the cutaneous lesions almost completely resolved after 15 weeks of treatment. Whether multicentric reticulohistiocytosis without arthritis is a subtype with better response to treatment remains to be confirmed by more clinical observation, but systemic corticosteroids may be tried first before starting other immunosuppressive or cytotoxic drugs.