Orbital Rosai-Dorfman disease treated with intralesional corticosteroid injection with 4-year follow-up

We describe a case of isolated orbital Rosai-Dorfman disease. Diagnostic uncertainty meant that two biopsies were required to exclude orbital lymphoma and immunoglobulin G4 (IgG4)-related disease. In accordance with histopathological guidelines, we present salient IgG4 parameters. Initial treatment involved oral corticosteroids and an excisional biopsy which led to resolution of symptoms; however, recurrence occurred 4 months later. Avoiding further surgery and in line with the patient’s preference, an intralesional corticosteroid injection was performed. The patient remains symptom-free after 4 years. This case and other cases reviewed here support the consideration for the early use of intralesional corticosteroids, a strategy which may avoid more toxic and resource intensive systemic treatments such as chemotherapy or immunomodulatory therapy.