Intralymphatic histiocytosis is characterized by dilated vessels containing aggregates of mononuclear histiocytes in their lumina, and the majority of cases have been associated with rheumatoid arthritis (RA). We report a case of a 61-year-old woman with RA who presented with livedo-like erythema and red papules around the right elbow and forearm. Histological examinations demonstrated numerous dilated vessels in the dermis partially filled with medium- to large-sized mononuclear cells. Immunohistochemically, most of the intraluminal cells were CD68-positive histiocytes. Endothelial cells were positive for D2-40, a marker for lymphatic endothelial cells. We diagnosed the patient with intralymphatic histiocytosis based on these clinical and histological findings. Interestingly, the skin manifestations exacerbated during the course of the disease when the swelling of the patient’s right elbow joint progressed. Then, the puncture of content fluid of the joint was performed, since then, the symptoms of the skin lesion immediately improved. These observations suggest a possible association between the eruptions of intralymphatic histiocytosis and the involved joint. The results suggest that such skin eruptions may be ameliorated by treating severe arthritis.