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Late‐onset neurofibromatosis in a liver transplant recipient
Author(s) -
Miller Martin B.,
Tonsgard James H.,
Soltani Keyoumars
Publication year - 2000
Publication title -
international journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.677
H-Index - 93
eISSN - 1365-4632
pISSN - 0011-9059
DOI - 10.1046/j.1365-4362.2000.00841-5.x
Subject(s) - medicine , nodule (geology) , eosinophilic , pathology , anatomy , physical examination , neurofibromatosis , papule , lesion , radiology , paleontology , biology
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 café‐au‐lait macules. Ophthalmic examination was remarkable for Lisch nodules ( Fig. 2). 1A representative clinical lesion as a fleshy, non‐tender nodule on the neck. Also a few very small lesions are barely visible on the mandible line2Iris hamartomas (Lisch nodules) in our patient 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 loosely 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). 3Histopathologic features of a cutaneous nodule demonstrating normal epidermis overlying a non‐encapsulated dermal mass (left panel), with loosely‐textured thin wavy eosinophilic fibers and strands, containing cells with spindle‐shaped or oval nuclei (right panel) 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.