ISSN:
1365-4632
Quelle:
Blackwell Publishing Journal Backfiles 1879-2005
Thema:
Medizin
Notizen:
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 ( 〈link href="#f5-1"〉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 ( 〈link href="#f5-2"〉Fig. 2).〈figure xml:id="f5-1"〉1〈mediaResource alt="image" href="urn:x-wiley:00119059:IJD841-5:IJD_841_f5-1"/〉A representative clinical lesion as a fleshy, non-tender nodule on the neck. Also a few very small lesions are barely visible on the mandible line〈figure xml:id="f5-2"〉2〈mediaResource alt="image" href="urn:x-wiley:00119059:IJD841-5:IJD_841_f5-2"/〉Iris hamartomas (Lisch nodules) in our patientTwo 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 ( 〈link href="#f5-3"〉Fig. 3).〈figure xml:id="f5-3"〉3〈mediaResource alt="image" href="urn:x-wiley:00119059:IJD841-5:IJD_841_f5-3"/〉Histopathologic 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.
Materialart:
Digitale Medien
URL:
http://dx.doi.org/10.1046/j.1365-4362.2000.00841-5.x
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