Cystinosis and gingival hyperplasia: Demonstration of cystine crystals in gingival tissue and unusual aspects of management
This is a case report concerning the management and pathological findings of a patient with cystinosis who developed gingival overgrowth secondary to the use of cyclosporine, which is the medication to prevent rejection of his kidney allograft. The patient was a 24year-old male with massive gingival overgrowth. The hyperplastic gingiva was causing extreme difficulty in eating swallowing and speaking. His current medications include phosphocysteamine 6 ml four times a day, cyclosporine-A 300 mg a day, prednisone 70 mg every other day, and levothyroxine 0.75 mg a day. The patient was to undergo periodontal surgery as an inpatient. Since he was on steroids, he was to be given 350 mg preoperatively of intravenous hydrocortisone. The patient developed oral candidiasis on the second postsurgical day. This was treated with clotrimazole troches with rapid resolution. At one week postoperatively, significant improvement was noted in oral function and gingival appearance. When the gingival tissue was fixed for observation under light microscopy, cells in the gingival connective tissue exhibited clear crystalline-like inclusions. Ultrathin sections showed the characteristic intracellular crystals described in other organs of cystinosis patients. The metabolic defect exists in all tissues of these patients; however, damage occurs to different tissues at different rates. The first organ to fail is the kidney. These patients have disturbances of growth. Later on, there are visual difficulties. Crystals accumulate in the cornea and other eye tissues, leading to photophobia and, eventually, visual acuity problems and even blindness. With survival, other organs fail later in the disease course, including thyroid, pancreas, muscle, and neurologic tissue. [M.C.R.]
Heller, A., D. Heller, A. Schwimmer, R. Gordon, and R. Cambria, J Periodont, 65:1139, 1994