Langerhans cell histiocytosis of the orbit 10 years after involvement at other sites

Langerhans cell histiocytosis within the orbit generally occurs in children and is marked by periorbital pain and swelling. Although these lesions respond well to minimal local curettage and concomitant corticosteroid injection, this disorder can be extremely aggressive and destructive. Recurrences usually occur within 12 months to 18 months after diagnosis. Herein, we discuss a patient who initially had mastoid and maxillary lesions and developed unilateral orbital involvement with periorbital pain and edema after a 10-year disease-free interval, and review other cases of late recurrence. The patient's symptoms resolved after curettage and injection of intralesional steroids. Clinicians should be aware that Langerhans cell histiocytosis may reappear at other sites, including the orbit, even after several years of quiescence.