We found a match
Your institution may have rights to this item. Sign in to continue.
- Title
Late-onset neurofibromatosis in a liver transplant recipient.
- Authors
Miller, Martin B.; Tonsgard, James H.; Soltani, Keyoumars
- Abstract
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 undergone 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 was certain that the lesions had appeared 2—3 months after his liver transplant. Wood's lamp examination revealed several caf´-au-lait macules. Ophthalmic examination was remarkable for Lisch nodules (Fig. 2). Two skin nodules on contralateral sides of the body were biopsied. Histologic examination of the nodules revealed a normal epidermis and a nonencapsulated dermal mass composed of thin, wavy eosinophilic fibers lying in Ioosely textured strands. Interdispersed among these strands were cells with spindle-shaped or oval nuclei and an increased number of mast cells. The histology was typical of a neurofibroma (Fig. 3). Computed tomography (CT) scans of the chest, abdomen, and pelvis taken before and after the transplant surgery did not reveal any internal plexiform neurofibromas. A thorough review of the patient's medical record dating to shortly after birth 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. Currently, the patient is on hemodialysis with no clinical progression of his neurofibromatosis.
- Subjects
NEUROFIBROMATOSIS; LIVER transplantation; CUTANEOUS manifestations of general diseases; ETIOLOGY of diseases; IMMUNOLOGY
- Publication
International Journal of Dermatology, 2000, Vol 39, Issue 5, p376
- ISSN
0011-9059
- Publication type
Article