BACKGROUND: Rosai-Dorfman disease is a rare disorder that was previously described as sinus histiocytosis with massive lymphadenopathy. The disease is derived from overproduction of monocytes, which play an important role in immunity. The overproduction of macrophages will lead to accumulation of the cells in the affected tissue.

CASE REPORT: A 40-year-old Saudi male presented with shortness of breath with joint pain starting 3 months prior. His main complaints were a dry cough, bilateral neck swelling, dry mouth, dry skin, itchy eyes, and general fatigability. Physical examination showed that the patient had prominent bilateral parotid swelling that measured roughly 5 by 3 cm, which was firm and tender, with a smooth surface and no local signs of inflammation. Joint examination revealed non-tender and non-swollen joints, with mild limitations of movement. Eye examination revealed dry eyes after having a positive Schirmer test. For diagnosis, the patient underwent complete blood count, autoantibody, histopathology, immunohistochemistry, and radiological assessment. The histopathological study confirmed Rosai-Dorfman disease. Rosai-Dorfman disease can involve various presentations, as in this patient, who exhibited a highly unusual presentation in association with autoimmune disease.

CONCLUSIONS: Rosai-Dorfman disease must be considered as differential diagnosis in patients who present with bilateral lymphadenopathy with multisystem complaints, as the disease can present with various characteristics.

Keywords: Autoimmune Diseases, Histiocytosis, Sinus, sarcoidosis, Sjogren's Syndrome, Arthralgia, Cough, Dyspnea, Edema, Fatigue, Xerostomia