Late-onset neurofibromatosis in a liver transplant recipient

A 44-year-old Caucasian man was admitted in December 1995 for the treatment of generalized skin pruritus. The patient had a history of cirrhosis felt to be caused by chronic ethanol abuse and renal insufficiency. He had under gone orthotopic liver transplantation in January 1991. Physical examination revealed numerous, fleshy, nontender nodules protruding from the face (Fig . 1), trunk, and extremities, as well as axillary freckling. The patient wa s certain that the lesions had appeared 2-3 months after his liver transpla nt. Wood's lamp examination revealed several cafe-au-lait macules. Ophthalm ic examination was remarkable for Lisch nodules (Fig. 2). Two skin nodules on contralateral sides of the body were biopsied. Histolog ic examination of the nodules revealed a normal epidermis and a nonencapsul ated dermal mass composed of thin, wavy eosinophilic fibers lying in loosel y textured strands. Interdispersed among these strands were cells with spin dle-shaped or oval nuclei and an increased number of mast cells. The histol ogy was typical of a neurofibroma (Fig. 3). Computed tomography (CT) scans of the chest, abdomen, and pelvis taken befo re and after the transplant surgery did not reveal any internal plexiform n eurofibromas. A thorough review of the patient's medical record dating to shortly after b irth made no mention of any skin nodules or hyperpigmentation prior to the transplant. There was no family history of neurofibromatosis. The patient's pruritus resolved with UVB light therapy and hemodialysis. Cu rrently, the patient is on hemodialysis with no clinical progression of his neurofibromatosis.